Mustafa Argun

Cheatham platinum stent implantation in children with coarctation of the aorta: single-centre short-term, intermediate-term, and long-term results from Turkey.

OBJECTIVES Our aim was to evaluate patients who were treated by percutaneous stent implantation. METHODS Patients with aortic coarctation (n = 35) who had been treated with 38 stents - 12 bare and 26 covered - were evaluated. The demographics and procedural and follow-up data were recorded from hospital registers and compared according to patient specifications, for example, weight and coarctation nature. RESULTS The procedure was successful in all patients. There was a statistically significant difference between the patients with native coarctation (n = 17) and those with recurrent coarctation (n = 18) in terms of pre-procedural blood pressures, systolic gradients, coarctation diameters, and the ratio of the coarctation site diameter to the descending aorta diameter. Although all patients received antihypertensive drugs before the procedure, the drug was discontinued in 26 patients during follow-up (p < 0.001). Stent migration was observed in four patients with recurrent coarctation (11.4%), and peripheral arterial injury was seen in three patients (8.5%). The mean follow-up time was 34 ± 16 months. On average, 21 (6-42) months after the procedure, six patients underwent cardiac catheterisation. At least 2 years after the procedure, tomography was performed in 20 patients (57.2%). Patients who were evaluated by multi-slice computerised tomography revealed no pathologies. There was no statistically significant difference between the five patients weighing less than 20 kg and the other 30 patients in terms of demographic and procedural characteristics, procedure success and complication rates, and follow-up data. CONCLUSION Stent implantation for aortic coarctation is a method yielding satisfactory results in reducing coarctation gradients, efficient enlargement of the lesion area, and resolution of hypertension for children, including those weighing less than 20 kg.

Different clinical presentations of Naxos disease and Carvajal syndrome: Case series from a single tertiary center and review of the literature.

Objective Naxos disease is an autosomal recessive, inherited, cardiocutaneous disorder, characterized by arrhythmogenic right ventricular cardiomyopathy, woolly hair, and palmoplantar keratoderma. Carvajal syndrome is characterized by palmoplantar keratoderma, curly hair, dilated cardiomyopathy, especially on the left ventricle side, and early morbidity. The aim of this study was to evaluate the cutaneous and cardiac findings and genotype-phenotype relationship of six patients diagnosed with Naxos/Carvajal syndrome. Methods A retrospective review of six cases diagnosed with Naxos/Carvajal syndrome at our institution from 2002 to 2012 was performed. Demographic data; presenting complaints; cutaneous and cardiac findings; electrocardiography, echocardiography, and genetic analysis results; and treatment data were obtained from patient files. Results The patient group was composed of 4 males and 2 females, ranging from 1.5 to 13 years, with a mean age 6.4 years. Typical cutaneous and hair findings were present in all patients. Two cases presented with ventricular tachycardia attack, and 2 cases presented with severe heart failure. Two cases had only cutaneous findings without cardiac involvement at diagnosis. An implantable cardioverter-defibrillator was implanted in one case due to ongoing recurrent ventricular tachycardia attacks despite various antiarrhythmic treatments. Three of the 6 patients died during the follow-up. Conclusion For cases with woolly hair and palmoplantar keratoderma, the physician should provide a cardiac assessment, considering Naxos/ Carvajal disease associated with cardiomyopathy. When an early diagnosis is made, the life expectancy may be increased by treatment of heart failure and arrhythmias; also, genetic counseling should be performed.

ADO II in Percutaneous VSD Closure in Pediatric Patients.

OBJECTIVES Main aim of our study to show that ADO II device can be used for the small ventricular septal defects successfully and safely with low complication rates in pediatric population. BACKGROUND It is hard to find an ideal device to use for every VSD successfully. If inappropriate device was chosen; complication rate increases, procedure time gets longer that prolongs exposure to ionizing radiation. Therefore interventionalists are in the search for new ideal devices. MATERIAL Between the dates April 2011-October 2014, 21 VSD closures with ADO-II device. were performed. Twenty patients were included, age ranged between 4 months 18 years. Weight of the patients was between 5-76 kg. RESULTS VSD diameter ranges between 2-6 mm (3.75 ± 1.25). VSD types were muscular in 2 patients, rest of them were perimembranous type. Most of the perimembranous defects (19/21) were aneursymatic and tunnel shaped. All the cases were successfully closed, no major complications were reported. There was no incidence of left bundle branch block, P-R prolongation, or complete heart block. CONCLUSION Considering perimembraneous ventricular septal defects as difficult and risky for percutaneous closure because of its proximity to aortic, atrioventricular valves and conduction tissue, we suggest that ADO II device can be safely and effectively used for such defects in particular if an aneurysm formation is present which is also compatible with the literature.

Plasma gelsolin as a biomarker of acute rheumatic carditis.

BACKGROUND Acute rheumatic fever is an autoimmune, inflammatory, and multi-systemic disease secondary to pharyngitis and is caused by group A streptococcus. In developing countries, acute rheumatic fever is the most common cause of acquired heart disease. Gelsolin is a calcium-dependent, multi-functional actin-regulatory protein circulating in the plasma of healthy human beings. The correlation between blood gelsolin levels and inflammatory conditions suggests the potential benefit of gelsolin as a prognostic marker. The aim of the present study was to appraise the association of gelsolin and acute rheumatic carditis in childhood. MATERIALS AND METHODS Plasma gelsolin levels were measured and echocardiographic examinations were performed in patients (n=37) with acute rheumatic carditis and compared with those of age- and gender-matched healthy controls (n=24). RESULTS The plasma gelsolin levels in children with acute rheumatic carditis were significantly lower compared with controls (197±218 versus 322±255 mg/L, p=0.039). There was a significant correlation among gelsolin levels and the grade of mitral regurgitation (p=0.030), left ventricular end-diastolic diameter (p=0.017), and left ventricular end-systolic diameter (p=0.028) at diagnosis. CONCLUSIONS Levels of the gelsolin plasma isoform were decreased in patients with acute rheumatic carditis compared with healthy controls. Gelsolin may be used as a biochemical marker for acute rheumatic carditis.

Efficacy of palivizumab prophylaxis among infants with congenital heart disease: A case control study

Palivizumab prophylaxis for RSV has been consistently reported to reduce the risk of hospital admissions related to RSV infection in children with symptomatic cardiac disease. This study was designed to investigate the efficacy of palivizumab prophylaxis among infants with congenital heart disease (CHD) in Turkey.

Do we need a femoral artery route for transvenous PDA closure in children with ADO-I?

Objective: The standard procedure in percutaneous closure of patent ductus arteriosus (PDA) with Amplatzer duct occluder-I (ADO-I) is transvenous closure guided by aortic access through femoral artery. The current study aims to compare the procedures for PDA closure with ADO-I: only transvenous access with the standard procedure. Methods: This study was designed retrospectively and 101 pediatric patients were included. PDA closure was done by only femoral venous access in 19 of them (group 1), arterial and venous access used in 92 patients (group 2) between 2004 to 2012 years. The position of the device and residual shunt in group1 was evaluated by the guidance of the aortogram obtained during the return phase of the pulmonary artery injection and guidance of transthoracic echocardiography. Shapiro-Wilk’s test, Mann-Whitney U, chi-squared tests were used for statistical comparison. Results: The procedure was successful in 18 (95%) patients in group 1 and 90 (98%) patients in group 2. Complications including the pulmonary artery embolization (n=1), protrusion to pulmonary artery (n=1), inguinal hematoma (n=3), bleeding (n=2) were only detected in group 2. In other words, while complications were observed in 7 (7.2%) patients in group 2, no minor/major complication was observed in group 1. Complete closure in group 1 was: in catheterization room 14 (77.8%), at 24th hour in 2 (11.1%), at first month in 2 (11.1%). Complete closure in group 2 was: 66 (73.4%) patients in the catheterization room, 21 (23.3%) at 24th hour, 3 (3.3%) at first month, complete closure occurred at the end of first month. Conclusion: In percutaneouse PDA closure via ADO-I, this technique can be a choice for patients whose femoral artery could not be accessed, or access is impossible/contraindicated. But for the reliability and validity of this method, randomized multicenter clinical studies are necessary.

Variable clinical presentation in primary lymphoedema: report of two cases.

Lymphoedema is a condition of localized fluid retention and tissue swelling caused by a compromised lymphatic system. Lymphoedema may be primary or secondary and can be inherited. Primary lymphoedema (primary lymphatic dysplasia) is a chronic oedema caused by a developmental abnormality of the lymphatic system. Primary lymphoedema most commonly affects the lower limbs, but other body parts can also be affected. It can be associated with some specific syndromes (i.e. Hennekam syndrome) and genetic disorders. In this article, we report on two patients with congenital multisegmental lymphoedema and Hennekam syndrome, both primary lymphoedemas.

Percutaneous atrial septal defect closure by using jugular venous access in a case with interrupted inferior vena cava

Femoral venous approach is the classical route of percutaneous atrial septal defect (ASD) closure. But in certain circumstances alternative routes are used. In this report percutaneous ASD closure in a case with interrupted vena cava by jugular venous approach is discussed. Percutaneous closure through femoral venous route was planned in a 6-year-old girl with ASD. Because of interrupted vena cava the jugular venous route was used. Having knowledge of this anatomical variation is important for interventionalists before performing femoral venous approach. Percutaneous transjugular venous access is a feasible alternative route in paediatric population for ASD closure.

Hypertension Associated with Coarctation of the Aorta Revisited: Case-Based Update from Experience of Three Children

Coarctation of the aorta (CoA) can present with different clinical pictures depending on the severity of the narrowness in the coarcted aortic segment in an age range between newborn and adolescence. Sometimes, it can cause intracranial hemorrhage or infarction when diagnosis and treatment are delayed. The aim of this report is taking attention to CoA as a cause of systemic hypertension and is also emphasizing the differences of diagnostic approach for hypertension in children from adults. Two cases of hypertensive cerebral hemorrhage and one case of hypertensive cerebellar infarction associated with CoA are reported. These cases help us to pay attention to the possibility of CoA in adolescents with hypertensive stroke. We want to emphasize the importance of physical examination for evaluation of hypertension and to impress the diagnostic approach for secondary hypertension in children.

Patent Ductus Arteriosus closure in preterms less than 2 kg: Surgery versus transcatheter

BACKGROUND As new devices come into the market, percutaneous techniques improve and interventionalists become more experienced; percutaneous closure gets more common in preterms. In this study we aimed to compare efficacy and safety of Patent Ductus Arteriosus closure surgically versus transcatheter method in preterms <2kg. Best of our knowledge this study is the first one that compares outcomes of surgery and percutaneous Patent Ductus Arteriosus closure in preterms. METHODS & RESULTS Between the dates July 1997 to October 2014 in our center Patent Ductus Arteriosus of 26 patients <2kg were closed percutaneously (Group A) and 31 less than 2kg operated (Group B). Weight of patients in percutaneous Patent Ductus Arteriosus closure group was significantly more than the surgery group. Mean gestational age of the patients in Group A was 30±1.8weeks, in group B was 28.6±3.5weeks. In group A; all cases were closed successfully except 4 cases: device embolization in 2, cardiac tamponade and iatrogenic aortic coarctation were seen. Pneumomediastinum and chylothorax were the major complications of the surgery group. There was no statistically significance between complication and success rates between two groups. CONCLUSION Percutaneous Patent Ductus Arteriosus closure is the candidate for taking the place of surgery in preterms. However, it is not applied routinely; can only be done in fully equipped large centers by experienced interventionalists.