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Objective: Dilated cardiomyopathy (DCM) is a disorder featuring left ventricular dysfunction, heart failure, and a poor prognosis. The etiology is still unclear, despite diagnostic and therapeutic developments. This study was an evaluation of factors affecting the life span of a group of idiopathic DCM patients., Methods: A total of 79 patients from between October 2005 and October 2017 with a diagnosis of idiopathic DCM were evaluated retrospectively. Demographic characteristics, clinical information, left ventricular function, treatment, and follow-up of the patients were reviewed based on hospital records. Age, gender, parental consanguinity, cardiomegaly on telecardiography, reduced ejection fraction (EF) and shortening fraction (SF), degree of mitral regurgitation, and intracardiac thrombosis were determined to affect prognosis., Results: The patients were aged 20+-60 months, and the male/female ratio was 1.02/1. The patients most frequently presented with heart failure signs and symptoms (n=59, 74.7%). The most common physical examination findings were a murmur (n=53, 67.1%) and tachycardia (n=48, 60.8%). Cardiomegaly was observed on telecardiography in 73.4% of the patients. The EF and SF values were 35.7+-12.6% and 17.3+-6.5%, respectively. In all, 42 (53.2%) patients had mitral regurgitation of grade 2 or higher. The duration of follow-up was between 1 and 156 months (20+-34.9 months). Intracardiac thrombosis was detected in 4 (5.1%) patients. The mortality rate was 36.7%. When the prognostic factors were compared according to survival time, it was determined that survival was reduced in cases of parental consanguinity, low EF, and cardiomegaly., Conclusion: The most important negative markers affecting the length of survival of DCM patients were parental consanguinity, cardiomegaly detected on telecardiography, and a reduced EF level.

Kommerell diverticulum is a rare phenomenon: Aberrant left subclavian artery in association with right aortic arch and ventricular septal defect. Kommerell's diverticulum can occur in a number of anomalies of the aortic arch system that can, but do not always, cause symptoms of tracheal or esophageal compression. The diverticulum is most frequently present in cases of right aortic arch with an aberrant left subclavian artery. In this anomaly, the left subclavian artery arises from the right-sided aortic arch as the 4th branch and extends behind the esophagus to the left arm. The descending aorta can be right sided or left sided. We report a 5-year-old girl with Kommerel diverticulum, minor left upper abnormality and restrictive perimembraneous ventricular septal defect. [ABSTRACT FROM AUTHOR]

Pulmonary atresia (PA) and ventricular septal defect (VSD) can occur in a variety of ways, from simple valve atresia to a condition in which circulation to the pulmonary bed occurs through collateral arteries separated from the aorta and there are no real pulmonary arteries, or they are present but hypoplastic. The size of the pulmonary arteries and concomitant complex cardiac lesions are important in making decisions about treatment and correctional alternatives. While complete correction surgeries in the style of a correction of tetralogy of Fallot are performed in simpler cases, many very invasive procedures are also performed and the resulting quality of life is very variable. The size of the pulmonary artery and its branches and the presence of accompanying collateral vessels are determining factors in the management of the disease. In this report, 2 cases of VSD and PA, in which the circulation of the pulmonary arteries was through the coronary arteries, diagnosed as a result of echocardiography performed following murmurs heard during examination, are described. The first patient was 40 days old and the second was 2 days old. In the second case, diagnosis was confirmed by catheter angiography, and in the first case, the final diagnosis was made during surgery. The aim of this report is to emphasize the importance of pulmonary artery size, the presence of pulmonary confluence, the origin of circulation, and concomitant collateral arteries when considering treatment methods.

Pulmonary arteriovenous malformation, which is defined as the presence of an ab-normal connection between the pulmonary artery and pulmonary vein, is rarely seen. Although it generally presents as a congenital condition, it may be accompanied by hereditary hemorrhagic telangiectasia. Clinical signs vary according to the amount of shunt in proportion to the number and size of the fistulae. Patients may present with cyanosis and respiratory trouble. If the disease remains untreated, it may result in cardiac failure and ineffective endocarditis, thereby leading to the rupture of the an-eurysmal fistula. Transcatheter embolization of abnormal vascular connection is the current treatment method in this disease. This article describes the case of an 8½-year-old child. He was presented with the symptom of getting tired quickly. Transcutaneous oxygen saturation of 75%, and pulmonary arteriovenous malfor-mation was detected in his examination. Successful transcatheter fistula embolization was performed.

Carpenter syndrome (Acrocephalopolysyndactyly type 2, OMIM 201000) is a rarely seen autosomal recessive disorder. In addition to abnormalities such as acrocephaly, craniosynostosis, facial asymmetry, polydactyly and syndactyly, obesity, hypogonadism, mental retardation, and corneal opacity, it may frequently be accompanied by congenital heart diseases such as ventricular septal defect, patent ductus arteriosus and pulmonary stenosis. Double outlet right ventricle is a defect in which both major arteries originate in the morphological right ventricle. To the best of our knowledge, this is the first report in the literature of double outlet right ventricle disease in combination with Carpenter syndrome.

Patients with wide patent ductus arteriosus and significant pulmonary hypertension not treated in time constitute a significant problem for cardiologists. For these patients, tests that could aid in decision-making for further planning include reversibility and balloon occlusion tests performed in the catheterization laboratory. Devices developed for the closure of ductus as well as different devices with off-label use may be employed in patients scheduled for transcatheter occlusion. When result of reversibility test is borderline positive, the use of fenestrated device may be applicable for selected patients. Presently described is case of a 10-year-old patient with Down syndrome who had a wide ductus and systemic pulmonary hypertension. Transcatheter closure procedure was performed with off-label use of a fenestrated muscular ventricular septal defect occluder device.

Objectives: The aim of this study was to share our experience in the diagnosis and treatment of patients who presented at our clinic with syncope, pre-syncope, dizziness, and palpitations., Study Design: Patients who were treated at pediatric cardiology clinic for complaints of syncope, dizziness, and palpitations between 2014 and 2016 were enrolled in the study. Detailed history of the patients, physical examination findings, laboratory and electrocardiogram results were recorded. Tilt table test, 24-hour Holter rhythm monitoring, and exercise test were performed, as required. Patients were diagnosed as vasovagal syncope, postural orthostatic tachycardia syndrome (POTS), or inappropriate sinus tachycardia based on these findings. Treatment of the patients was evaluated., Results: Thirty patients were diagnosed as vasovagal syncope, 7 patients as POTS, and 2 as inappropriate sinus tachycardia. POTS accompanied Raynaud's phenomenon in 1 patient, hypertrophic cardiomyopathy in 1 patient, and homocystinuria in another patient. Complaints of patients with vasovagal syncope improved with non-medical therapy. Medical treatment was administered to the patients with diagnosis of POTS and inappropriate sinus tachycardia., Conclusion: In patients with complaints of syncope, pre-syncope, dizziness, and palpitations without structural heart disease or non-rhythm problems, cardiovascular autonomic disorders, such as POTS and inappropriate sinus tachycardia should be kept in mind, as well as vasovagal syncope.

Atrioventricular (AV) block in the neonatal period is a rare disorder. It is frequently associated with underlying structural congenital heart disease and maternal lupus. Presently described is premature baby who developed 2:1 AV block and congestive heart failure due to hypocalcemia. Dramatic clinical improvement was observed following treatment of intravenous 10% calcium gluconate. Therefore, it is suggested that serum calcium level of newborns with AV block and congestive heart failure be measured.

Balloon valvuloplasty is an effective therapy for severe congenital aortic valve stenosis, with mild aortic insufficiency and minimal intermediate-term restenosis. No consensus currently exists regarding optimal vascular approach for balloon dilatation in newborns with critical or severe aortic valve stenosis. Critical aortic valve stenosis in newborns must be treated promptly and effectively. Transcatheter therapy may offer marked advantages, as surgical therapy has been associated with significant rates of morbidity and mortality. Percutaneous balloon dilatation is usually performed as emergent therapy of valve stenosis, with various options for vascular approach. While umbilical artery and vein access is rarely used in the treatment of critical aortic valve stenosis and aortic coarctation, this approach is a safe, simple, and effective choice for balloon dilatation in newborns, even in those weighing under 2.5 kg.

Amaç: Selenyum bir çok enzimin kofaktörüdür ve temel olarak antioksidan fonksiyonuyla bilinen esansiyel bir eser elementtir. Serum selenyum düzeyi cinsiyet, yaş, sigara ve çevresel faktörlerden etkilense de diyetle alınan selenyumun en temel göstergesidir. Çalışmamızın amacı hastanemizin Kadın Doğum Servisinde dünyaya gelen sağlıklı bebeklerin serum selenyum düzeylerinin tespiti ve cinsiyetler arası farklılığın olup olmadığını incelemektir. Yöntem: Çalışmaya 95 (4-109) günlük 34 erkek ve 94 (4-110) günlük 35 kız olmak üzere toplam 69 bebek dâhil edildi. Bebeklerin serum selenyum düzeyleri Zieman düzeltmeli atomik absorbsiyon spektroskopisi (Varian GTA120\\ AA240Z) kullanılarak ölçüldü. Bulgular: Araştırmaya katılan bebeklerin serum selenyum median (min-max) düzeyleri 53,4 (16,35-149,25) µg/L olarak tespit edildi. Erkek bebeklerin serum selenyum düzeyleri 52,7 (24,75-149,25) µg/L ve kız bebeklerin serum selenyum düzeyleri 53,4 (16,35-102,30) µg/L olarak tespit edildi. Cinsiyetler arası serum selenyum düzeyleri arasında anlamlı bir fark tespit edilmedi. Sonuç: Plazma selenyum düzeyi sağlıklı neonataller de serum selenyum düzeyi < 8 µg/L ve < 2 yaş bebeklerde 16-71 µg/L aralığında değişmektedir. Bizim çalışma verilerimiz literatür bilgileriyle uyumlu bulunmuştur. [ABSTRACT FROM AUTHOR]

Costello syndrome is a rare syndrome characterized by failure to thrive, short stature, mental motor retardation, characteristic facial features, macrocephaly, a short neck, loose soft skin with deep palmar and plantar creases, and hypertrichosis. Cardiac involvement is seen in almost two thirds of patients, and is a determinant for the prognosis of Costello syndrome. The most common cardiac anomalies are pulmonary stenosis, hypertrophic cardiomyopathy, atrial septal defect, ventricular septal defect and arrhytmia. In this report, we present a 14-month-old female pediatric patient with hypertrophic cardiomyopathy, clinically and genetically diagnosed with Costello syndrome. The report also contains a review of recent related literature.

Turkey's institutions in cardiovascular medicine were evaluated regarding publication output in 2013 based on data available in the Web of Science. Only articles in full-text and reviews appearing in source publications covered by the Science Citation Index proper were included. A fractional count system was used for items published jointly with a foreign or a non-cardiological Turkish institution. Turkey's publications increased numerically to 268, and its global share from 4.3 to 6.0 per mille, yet only to the level of 2001. Articles originating from adult cardiology numbered 188 (70%), while cardiovascular surgery and pediatric cardiology contributed 15% each. Three hospitals affiliated with the Ministry of Health (Kartal Koşuyolu, M. Akif Ersoy and Türkiye Yüksek İhtisas Hastanesi) and the cardiology departments of the medical faculties of Başkent, Hacettepe and Ege Universities ranked highest, each generating 7-15 papers. The median impact factor remained similar to the preceding year, at 1.52 (interquartile range 1.20-2.31). The undertaking of promotional and supportive measures by the central authorities regarding the conspicuous decline in Turkey's medical research output is long overdue.

Objectives: In this study, we evaluated the correlation between severity of deformity and cardiopulmonary function with regards to echocardiographic and spirometric findings., Study Design: Twenty-five children, mean age 13.6 years, presenting with pectus excavatum between August 2012 and May 2013, were included. Haller index (HI) was calculated for each patient. Patients with an index of <2.5 were accepted as Group 1, 2.5-3.6 as Group 2, and >3.6 as Group 3. Left ventricle dimension, ejection fraction, and shortening fraction were evaluated with echocardiography. Using spirometry, forced vital capacity (FVC), forced expiratory volume in the first second (FEV1), and FEV1/FVC ratio were calculated. Groups were compared using these parameters., Results: There were 18 males and 7 females. The mean index was 3.48±0.78. Though there was no significant difference in the index with regards to sex, the index increased with age. Eight percent of patients were in Group 1, 52% in Group 2 and 40% in Group 3. A significant decrease in ejection and shortening fractions was evident as the index increased. A statistically significant relation between HI and cardiac dysfunction was evident (p<0.01). As the index increased, there was significant decrease in FEV1 and the FEV1/FVC ratio, while there was no significant difference in FVC. As the deformity worsened, incidence of pulmonary dysfunction was found to be higher., Conclusion: This study revealed that pectus excavatum leads to cardiac and pulmonary problems, and functions of the left ventricle may be affected by the deformity. Furthermore, the relation between the severity of the deformity and cardiovascular function is evident.

Objectives: We aimed to evaluate the short- and mid-term results of patients with atrial septal defect (ASD) who were treated with percutaneous closure., Study Design: Seventy-nine patients with secundum ASD (54 female and 25 male; mean age 26.2±17.2; range 3 to 71] years) were included in this study. Patients were evaluated by transthoracic (TTE) and/or transesophageal echocardiography (TEE). Amplatzer septal occluder (ASO) was used in all patients. In 76 patients, the procedure was performed under local anesthesia with TTE, while in the other 3 patients, it was performed with general anesthesia with TEE. Patients were followed up at the 1st, 3rd, 6th and 12th months and annually thereafter. Mean follow-up time was 13.6±6.6 months., Results: Mean diameter of ASDs was 18.2±7.5 mm and 20.7±8.04 mm during balloon dilatation, and mean diameter of implanted devices was 22.7±8.5 mm. Procedural time was 40.2±12.6 minutes and fluoroscopy time was 10.9±4.1 minutes. The procedure was successfully performed in all patients (100%). One patient with cardiac tamponade died seven days after cardiac surgery. In two patients, the implanted devices embolized to the pulmonary circulation. Residual flow was found in three patients immediately after the procedure, without residual shunts one month after closure. Mild pericardial effusion in one patient and significant residual shunt due to device malposition in another were discovered during the follow-up at 1 and 6 months, respectively, after the procedure., Conclusion: Our findings showed that percutaneous closure of ASDs is successful in most patients with a low complication rate, and demonstrated that residual shunts do not develop in the majority of patients in the short- and mid-term.

Posttraumatic pulmonary arteriovenous fistula is a rare complication of penetrating injury. Cases may remain asymptomatic for years prior to diagnosis. We present a case of pulmonary arteriovenous fistula related to a penetrating injury of the thorax 6 years previously that was occluded with a septal occluder device. Consistent use of CT or conventional catheter pulmonary angiography following penetrating injury to the thorax is essential to the early diagnosis of pulmonary arteriovenous fistula during the asymptomatic stage. Modern occlude devices facilitate the transcatheter approach as a viable alternative to surgery for the closure large, high pressure defects.

The insertion of ventriculoatrial (VA) shunts for the treatment of hydrocephalus is associated with the development of chronic thromboembolic pulmonary hypertension (CTEPH). Chronic thromboembolic pulmonary hypertension occurs in patients with recurrent or chronic pulmonary embolism, and is a rare but, potentially devastating disease in children. Pulmonary thromboendarterectomy (PTE) is an important curative therapy for patients with CTEPH. Herein, we present a case of a 14 year-old male patient with CTEPH that developed after a VA shunt procedure. After successful PTE, systolic pulmonary artery pressure was decreased from 75 mmHg to 30 mmHg. PTE is recommended in the pediatric CTEPH population.

A vascular ring is defined as an anomaly of the great arteries (aortic arch and its branches) that compresses the trachea or esophagus. Double aortic arch is the most common vascular ring. Double aortic arch is very rare and typically becomes symptomatic in infancy or early childhood. We present a 7-year-old girl admitted to our clinic for evaluation of recurrent respiratory infection with dysphagia. Double aortic arch was suspected from echocardiography and diagnosed with cardiac computed tomography. Left aortic arcus was larger than the right at computed tomography and cardiac catheterisation. After surgery the symptoms improved strikingly. We conclude that vascular ring should be considered in the patients presenting with recurrent pulmonary infections and dysphagia. Early diagnosis and treatment may prevent chronic, irreversible complications.

Objectives: The purpose of our study was to determine structural and functional changes on left ventricular function (LV) according to body mass index (BMI) in normotansive overweight and obese children., Study Design: Thirty normotansive overweight children (group 2; mean age: 13.2 ± 2.1 years, BMI: 25-30 kg/m²), 30 obese children (group 3; mean age: 13.3 ± 2.0 years, BMI ≥ 30 kg/m²), and 50 healthy controls (mean age: 13.2 ± 1.8 years, BMI: 18-24.9 kg/m²) were included in this study. Continuous ambulatory pressure was monitored in the obese group, while standard and pulsed wave (PW) Doppler echocardiographic examinations were evaluated in all three study groups., Results: In overweight and obese children, left atrial volume, left atrial/aortic root diameter ratio, LV interventricular septum, LV posterior wall thickness, LV end-diastolic diameter and volume, and LV mass were significantly higher than those children in the control group (p<0.01). Blood pressure was within the normal range but was increased in the obese groups. Transmitral E/A and pulmonary vein (PV) systolic/diastolic velocity (S/D) ratios were decreased, but E-wave deceleration time, PVA velocities, and the end-diastolic distance from the mitral annulus to the LV apex were increased in both obese groups (p<0.05). BMI was significantly correlated with duration of obesity and LV mass (r=0.527, r=0.506, p<0.01, respectively). Significantly negative correlations were found between BMI, Mitral E/A, and PV S/D ratio (r=-0.230, r=-0.577, p<0.01, respectively)., Conclusion: Subclinical LV myocardial dysfunction was noted in obese subgroups. Determination of diastolic dysfunction by PV PW Doppler can be useful a pre-obese period.

Objectives: To determine and disclose publications from Turkey that have most contributed "genuinely" to cardiovascular medicine in the past 40 years., Study Design: Based on data of Web of Science, 50 publications were identified, originating from Turkey's institutions and having received the greatest number of citations as of July 2011. Papers having more than a minor share by international authors were not considered., Results: Thirty-eight primary authors generated 50 medical papers, each receiving ≥37 citations (95% CI 37; 124). The herein cited articles corresponded in quality to the top one-fifth of global papers. Half of the articles were published in 1997-2003. Currently, the estimated number of such papers produced annually in Turkey is only 4-5, which represents only a global share of 1.0 per mille. The best period registered were the years 1995 to 2005, after which a rise is not anticipated to persist. Cardiology generated 29, cardiovascular surgery 19, and pediatric cardiology two articles. These publications originated from only 10 medical faculties, Gülhane Military Academy, three state and two private hospitals., Conclusion: Given its potential, the performance of Turkey's contribution to cardiovascular medicine has been unsatisfactory. A much larger concerted effort is required to form a milieu that will focus to enhance research having a potential to contribute to medicine.

Objectives: We evaluated long-term follow-up results and prognosis of pediatric patients with isolated ventricular septal defects (VSD)., Study Design: The study included 799 patients (368 girls, 431 boys; mean age at diagnosis 24.3±37.4 months; median 6 months) who were monitored by the pediatric cardiology department for VSD. The mean follow-up period was 32.8±30.3 months (median 20 months)., Results: The VSDs were classified as perimembranous (n=610, 76.4%), muscular (n=171, 21.4%), doubly committed subarterial (n=10, 1.3%), and multiple (n=8, 1%). Spontaneous closure rates were 42.7%, 13.1%, and 25% in muscular, perimembranous, and multiple VSDs, respectively, which corresponded to a mean age of 18.6±19.9 months (median 12 months) in muscular and 30.2±33.7 months (median 14.5 months) in perimembranous VSDs. Before 2 years of age, 78.1% of muscular and 58.6% of perimembranous VSDs underwent spontaneous closure. Of 256 defects (32%) that required surgical closure, 91.4% were of perimembranous location. The mean age at surgery was 38.8±49.1 months (median 11 months) for muscular, and 43.7±40.9 months (median 24 months) for perimembranous defects. During the follow-up period, the following complications were noted: aortic valve prolapse (0.7%), aortic regurgitation (0.6%), left ventricle-to-right atrium shunt (2.6%), subaortic ridge (3.7%), and infundibular stenosis (1.2%). Aortic regurgitation developed in eight patients (3.7%) after surgical closure., Conclusion: Our data on the natural course and prognosis of VSDs may be of relevance with respect to patients' age, defect type, and complications encountered in the follow-up period.

Scimitar syndrome is characterized by partial or total anomalous pulmonary venous return from the right lung. We present a 15-month-old boy who was diagnosed with Scimitar syndrome after examinations for recurrent wheezing and respiratory distress. The chest radiograph showed a scimitar sign in the right hemithorax, obscuring the contours of the right atrium. Echocardiography showed dilatation of the right heart cavities and increased flow in the inferior vena cava, without a cardiac abnormality. The patient underwent cardiac catheterization for radiographic and hemodynamic evaluations, during which a scimitar vein was detected, draining the right pulmonary veins to the inferior vena cava. Coil occlusion was performed on the abnormal artery arising from the infradiaphragmatic aorta. The patient was referred to surgery for repair of the anomalous pulmonary venous return and resection of the sequestered pulmonary segment.

Autosomal recessive dystrophic epidermolysis bullosa (DEB) is a chronic skin disorder characterized by widespread bullous formation, erosions, and scar formation. There have been reports of dilated cardiomyopathy and death in patients with DEB. The pathogenesis of cardiomyopathy in DEB remains uncertain, but some drugs, viral infections, iron loading, micronutrient deficiencies such as selenium and carnitine have been implicated. A 16-year-old boy who was followed-up from birth with the diagnosis of DEB presented with respiratory distress and heart failure symptoms of two-week history and early fatigue within the past year. Etiological evaluation showed a low plasma selenium level. Echocardiographic examination yielded the diagnosis of dilated cardiomyopathy. Findings of viral serology tests and metabolic screening were normal. Selenium replacement and anticongestive treatment were initiated, which led to partial improvement in cardiac functions. The authors draw attention to the possible role of micronutrient deficiency in the development of cardiomyopathy in patients with DEB.

Stent implantation into a ductus arteriosus or systemic-pulmonary shunt is a relatively new but infrequent approach for palliation. A 22-month-old boy with a modified Blalock-Taussig shunt for tetralogy of Fallot was admitted with severe cyanosis. Echocardiographic examination showed complete occlusion of the shunt. Initial balloon angioplasty resulted in reocclusion of the shunt by acute thrombosis. Finally, recanalization was achieved by stent implantation. Oxygen saturation of the patient increased from 32% to 92% following stenting. He was discharged on aspirin therapy.

Methemoglobinemia is characterized by varying degrees of cyanosis due to increased hemoglobin concentrations containing oxidized iron. Prilocaine is a widely used local anesthetic and can cause methemoglobinemia in infants even in therapeutic doses. We present two female infants (younger than 2 months) who developed severe cyanosis after transcatheter pulmonary balloon valvuloplasty and were diagnosed with toxic methemoglobinemia. Both infants were anesthetized with local prilocaine application before balloon valvuloplasty. Methemoglobin levels of the patients were measured as 49.6% and 37.7%, respectively. Both were successfully treated with intravenous methylene blue and ascorbic acid.

Arterial tortuosity syndrome (ATS) is a rare autosomal recessive connective tissue disease characterized by elongation, tortuosity, aneurysmatic formation, and stenotic lesions in large and medium-size arteries. We present two cases of ATS diagnosed during cardiac examination for murmurs. The first was an 11-year-old boy who had an atypical facial appearance and hyperelasticity. He had a prior operation for inguinal hernia. Echocardiography showed aneurysmatic dilatation in the main pulmonary artery and peripheral stenotic lesions. Angiography and computed tomography angiography confirmed aneurysmatic formation in the main pulmonary artery and multiple stenotic lesions in peripheral arteries and showed elongation and tortuosity of the major branches of the aorta. Surgical reconstruction of the pulmonary arterial system was performed. The second was a 3-month-old girl with an atypical facial appearance, hyperelasticity, and marked hypotonia. The aortic arch could not be visualized during echocardiography. Angiographic examination showed mild bilateral stenosis of distal pulmonary arteries, elongation and tortuosity of the aortic arch and its main branches.

Lipomatous hypertrophy of the interatrial septum is a rare benign pathology characterized by fatty deposits in the septum and is mostly diagnosed incidentally. This accumulation mostly causes a globular thickening of the interatrial septum, commonly sparing the fossa ovalis. We report on a 65-year-old female patient who underwent successful transcatheter closure of atrial septal defects (ASD) accompanied by lipomatous hypertrophy of the septum. Both transthoracic and transesophageal echocardiography showed enlargement of the right heart cavities, thickening of the interatrial septum (16 mm) with bright echogenicity, and two separate secundum ASDs measuring 17 mm and 4 mm, respectively. Transcatheter closure of the defects was performed using a 24-mm Amplatzer septal occluder. There was no residual shunt and Holter monitoring was normal after the procedure. During a three-year follow-up, no complications were observed pertaining to the procedure or lipomatous tissue.

Objectives: We investigated the incidence of unresponsiveness to intravenous gamma globulin (IVIG) treatment in Kawasaki disease (KD) and evaluated its relation with coronary artery involvement., Study Design: The study included 20 children (13 boys, 7 girls; mean age 4.2+/-3.4 years; range 9 months to 12 years) with KD. The mean disease duration on admission was 7.3+/-2.4 days (range 5 to 14 days). Initial treatment consisted of a single dose of IVIG and high-dose of aspirin. Unresponsiveness was defined as the persistence of fever and other symptoms within the first 48 hours of treatment. All the patients were evaluated by two-dimensional echocardiography before and after treatment. The mean follow-up period was 16.5+/-2.8 months (range 9 to 24 months)., Results: Unresponsiveness was seen in five patients (25%), who received a subsequent dose of IVIG, which improved fever in two patients. The remaining three patients received high-dose methylprednisolone. One patient who showed no response to either IVIG or methylprednisolone was treated with low-dose oral methotrexate. Six patients (30%) had coronary artery involvement (4 dilatations, 2 aneurysms), five patients on admission echocardiography, and one patient on control echocardiography seven days after treatment. Of five unresponsive patients, four (80%) had coronary artery involvement on admission. Patients with coronary involvement underwent coronary angiography after a mean of one year. Five patients had normal coronary arteries, whereas no angiographic regression was observed in one patient who had a giant coronary artery aneurysm on admission and was treated with oral methotrexate., Conclusion: The incidence of unresponsiveness to treatment was markedly high in KD patients who had coronary artery involvement on admission.

The output of publications in cardiovascular medicine during 2008 originating from Turkey's institutions were evaluated based on data of the Web of Knowledge. Only original articles and reviews appearing full-text in source publications of Science Citation Index CD Edition alone were included. A weighted credit system was used for items published jointly by multiple institutions or with a noncardiological department. Turkey's publications rose substantially to 220 articles and reviews, as her share of world publication rose from 10.0 to 10.8 per mille. Seven-eighths of the output originated from adult cardiology, pointing to a negative trend with respect to the fields of cardiovascular surgery and pediatric cardiology. Though the median impact factor was similar with 1.56 to that of the previous year, the finding that half of the publications appeared in periodicals with an impact factor of 1.19 to 2.88 showed a trend to target relatively better journals. Our established medical faculties, with the exception of the Aegean faculty, continued to lag behind in productivity, while the Başkent University led by far, and the Gülhane Military Medical Academy, Gazi University, and Siyami Ersek Cardiovascular Surgery Center were runners-up.

Coexistence of partial anomalous pulmonary venous drainage (PVD) and pulmonary atresia with ventricular septal defect (PA-VSD) is very rare with the exception of heterotaxy syndromes. We presented two cases of PA-VSD in which partial anomalous PVD was detected during pulmonary artery angiography. One was a six-month-old girl who underwent cardiac catheterization to evaluate the chance of unifocalization. There were true pulmonary arteries and three major aortopulmonary collaterals. It was noted that the right upper pulmonary vein was in direct continuity with the right atrium. The second case was a 12-year-old girl who was previously diagnosed as having PA-VSD and absence of true pulmonary arteries, for which she had undergone two subsequent aortopulmonary shunt operations four years before. During cardiac catheterization, confluent but hypoplastic true pulmonary arteries and multiple minor aortopulmonary collaterals were detected. The right and left upper pulmonary veins were draining to the right atrium. It should be known that, albeit very rare, partial anomalous PVD may be present in PA-VSD cases, requiring careful evaluation of pulmonary venous drainage during echocardiographic and angiographic studies.

Objective: Transesophageal echocardiography (TEE) is indicated for suspected atrial septal pathology and for monitoring of interventional procedures such as an atrial septal defect (ASD) closure during cardiac catheterization. Transesophageal echocardiography also helps to demonstrate postoperative complications and residual defects of complex congenital cardiac anomalies., Methods: Transesophageal echocardiography was performed in 112 pediatric patients with or suspected atrial pathology at our institution between 1999-2002, using the standard techniques. The mean age was 8.7+/-4.2 years., Results: In 45 of 112 children the suspected atrial defects were confirmed with the TEE. Patent foramen ovale was correctly predicted in 13.4% of patients by TEE, but only in 8.7% of patients by echocardiography. Multiple ASD's were correctly defined in 4.1%, and high venosus defects were documented in 6.1% of children by the TEE. We used TEE in 13% of patients for detecting atrial vegetations in patients with possible endocarditis, and evaluation of the postoperative care of atrial surgery such as Fontan or Senning operations and total correction of abnormal pulmonary venous return. Successful transcatheter closure of 7 ASD's was accomplished under TEE guidance., Conclusion: Transesophageal echocardiography allows a much more detailed evaluation of atrial morphology than transthoracic echocardiography even in infants. Transesophageal echocardiography is also indicated during interventional procedures and postoperative evaluation of the atrial pathology.

Objective: To evaluate patients who were referred to our center with suspicion of fetal arrhythmia for diagnosis, results of treatment and prognosis., Methods: Thirty-three patients referred to our center for evaluation of fetal cardiac arrhythmias were examined by M-mode, two-dimensional and Doppler echocardiography for cardiac anomaly and type of dysrhythmia, Results: Arrhythmias were diagnosed in 15 of 33 patients. Four cases demonstrated tachycardia, 5--ectopic beats, 6--bradycardia. Of 5 fetuses with tachycardia, transplacental antiarrhythmic drugs were administered in three cases; two of them with hydrops died and conversion of the arrhythmia was achieved in one. Six patients with bradycardia had poor perinatal outcome, with 3 deaths occurred (two of them had third degree atrioventricular block, one of them had sinus bradycardia). All patients with ectopic beats had a good perinatal outcome., Conclusion: The results suggest that bradycardic and/or hydropic fetuses have poor prognosis, however fetuses with ectopic beats generally have desirable prognosis.