Abdullah Kocabaş

Native Aortic Valve Endocarditis Complicated by Pseudoaneurysm of Mitral-Aortic Intervalvular Fibrosa

Pseudoaneurysm of mitral‐aortic intervalvular fibrosa (PA‐MAIVF) is a rare complication of native aortic valve endocarditis. This region is a relatively avascular area and prone to infection during endocarditis and subsequent aneurysm formation. The rupture into the pericardial cavity and left atrium or aorta, systemic embolism, myocardial infarction secondary to left coronary compression, and sudden death are the reported complications. Herein, we present a 9‐year‐old boy who was diagnosed with bicuspid aortic valve endocarditis complicated by PA‐MAIVF, cerebral embolism, and hemorrhage. PA‐MAIVF was visualized by both two‐ and three‐dimensional transthoracic echocardiography and ruptured into pericardial space causing a fatal outcome.

Cardiac Autonomic Dysfunction in Patients With Infantile Spasm and the Effect of Adrenocorticotropic Hormone Treatment

Infantile spasm is an age-dependent epileptic-encephalopathy syndrome. Cardiac autonomic function is frequently altered in epilepsy. In this study, we examined heart rate variability in patients with infantile spasm before and after treatment. Nineteen patients with infantile spasm and 13 healthy comparisons were enrolled in the study. Cardiac rhythm was recorded with a Holter device for 24 hours before adrenocorticotropic hormone (ACTH) (Synacthen depot) and B6 vitamin administration and 1 month after treatment. Heart rate variability analysis found lower heart rate variability parameters in patients with infantile spasm at the onset of the syndrome, prior to treatment with ACTH. The time domain parameters of heart rate variability values showed a statistically significant increase following ACTH treatment. Our data suggest that patients with infantile spasm exhibit lower heart rate variability parameters, and the treatment of spasms with ACTH and B6 together diminished the autonomic dysfunction in our cohort.

Is there any difference in clinical features of Turkish children with Kawasaki disease

Address for Correspondence: Dr. Filiz Ekici, Ankara Cocuk Hematoloji ve Onkoloji Egitim ve Arastirma Hastanesi, Pediyatri Kardiyoloji Klinigi, Ankara-Turkiye Phone: +90 312 596 96 56 E-mail: ekicifiliz@gmail.comAccepted Date: 21.04.2014 Available Online Date: 22.08.2014©Copyright 2014 by Turkish Society of Cardiology - Available online at www.anakarder.comDOI:10.5152/akd.2014.5629Article in Press

Intracardiac Thrombus in Children: The Fine Equilibrium Between the Risk and the Benefit

The medical records of 16 patients diagnosed as intracardiac thrombus were searched. The size, location and outcome of thrombus together with demographic data of patients were assessed. The median age of the patients was 2.2 years. Six patients were newborn and two patients were infant. The median size of thrombus was 9 mm. The localization was right atrium in seven, right ventricle in five, left ventricle in one, pulmonary artery in one, and superior vena cava in two patients. There was prematurity in five, ciyanotic congenital heart disease in one, blood culture positivity in three, malignancy in four, nephrotic syndrome in one, indwelling catheters in 10, and acquired or genetic thrombophilia in six patients as risk factors. In the treatment, the first choice was tissue plasminogen activator in two patients, heparin infusion in one patient and low molecular weight heparin in remaining 12 patients. In nine patients, therapy included parenteral antimicrobials together with anticoagulants. The result was complete resolution in 15 patients and in one patient thrombus was surgically removed. The median time was 16 (2–70) days for 50% resolution and 26 (3–93) days for complete resolution. There was a statistically significant (P = .027 and r = 0.5) correlation between the size and the complete resolution time. There was no anticoagulant therapy related major complication. In patients with intracardiac thrombus, selection of anticoagulant therapy may decrease the risk of complications. Surgery is rarely required and thrombolytics are not usually necessary for resolution of thrombus.

Facial Nerve Palsy and Kawasaki Disease

Kawasaki Disease (KD) is a vasculitic disease and can affect any organ system in the body. The development of coronary artery aneurysms is the most common and life threatening complication of KD and makes this disease the leading cause of acquired heart disease in children in the developed world. Facial nerve palsy has been reported as a possible marker of more severe disease and increased risk of coronary artery involvement in KD. Herein, the authors report an 8-mo-old infant who had left sided facial nerve palsy and multiple coronary aneurysms associated with KD.

Three-Dimensional Echocardiographic Evaluation of a Patient with Double-Orifice Mitral Valve, Bicuspid Aortic Valve, and Coarctation of Aorta

A 16-year-old male patient was diagnosed with bicuspid aortic valve and coarctation of aorta, and had a history of a subclavian patch plasty operation at 1 year of age. In addition, we detected double-orifice mitral valve (DOMV) at a routine evaluation and demonstrated by both two-dimensional and three-dimensional (3D) echocardiographic examinations. DOMV should be kept in mind in cases with left ventricular obstructive lesions and 3D echocardiography may provide a more detailed assessment of mitral valve and subvalvular apparatus.

Mid-Aortic Syndrome Demonstrated by Three-Dimensional Computed Tomography: Case Report

A 14-year-old male patient was admitted to our clinic for evaluation of systemic arterial hypertension. His right and left arm arterial blood pressures were extremely high and measured 200/120 and 180/120 mmHg, respectively. His bilateral femoral pulses were weak, and lower limb blood pressures could not be measured. By conventional echocardiography, increased left ventricular wall thickness, narrowed lumen size, and disturbed antegrade flow at the lower thoracic part of aorta were shown (Fig. 1, Movie clip 1). Three-dimensional computed tomography (CT) clearly demonstrated a long-segment severe coarctation involving the distal half of thoracic aorta (Fig. 2). The affected segment was 3 cm in length, and the intra-aortic lumen size decreased gradually to \95 % of the expected lumen size. Intrarenal part of the abdominal aorta was also narrowed, and there were no stenosis in the other branches of the aorta. Multiple aortico-aortic collaterals were also detected by CT evaluation. Cranial magnetic resonance imaging and ophthalmologic examinations were normal, and serum inflammatory markers were within the normal ranges. Thus, the patient was diagnosed with an idiopathic form of mid-aortic syndrome (MAS). Due to long-segment involvement of aorta, percutaneous transluminal angioplasty of the aorta could not be performed. We therefore planned aorta‐aortic bypass graft surgery for this patient. MAS is a rare congenital abnormality characterized by segmental narrowing of the abdominal or distal descending thoracic aorta [2‐5]. It is frequently seen with stenosis of the renal and mesenteric arteries. It can be acquired or congenital. Acquired forms are usually associated with neurofibromatosis, fibromuscular dysplasia, retroperitoneal fibrosis, Williams syndrome, mucopolysaccharidosis, and giant-cell arteritis [2, 4]. Three-dimensional CT can provide more detailed information regarding cardiovascular structures than conventional echocardiographic techniques. MAS can cause severe renovascular hypertension in children and adolescents. It is generally difficult to manage the hypertension which requires treatment with several

The efficacy and safety of naproxen in acute rheumatic fever: the comparative results of 11-year experience with acetylsalicylic acid and naproxen

This clinical study was designed to evaluate mortality rate and the factors that may affect survival in neonatal surgery unit. Randomly chosen 300 (ß: 0.20) patients among 1,439 patients treated in neonatal surgery unit during years 1983 to 2009, were evaluated retrospectively. The patients were separated into three groups according to date of treatment; Group A: 1983 - 1995, Group B: 1996 - 2005 and Group C: 2005 - 2009. M/F ratios did not differ between non-survived and survived patient populations. Mortality rates were 37%, 22% and 13% in Group A, B, and C respectively (p < 0.001). Parenteral nutrition, maternal age, time until admission and gestational age did not affect mortality rate, however median age of newborn was lower in non-survived cases (1 day vs. 3 days, p < 0.001). Associating abnormality, low birth weight ( < 1,500 g), associating sepsis, need of globulin and requirement of respiratory support were determinants of lower survival (p < 0.001). The mortality rate for patients that underwent thoracotomy (42%) and laparotomy (41%) were higher than patients that underwent other operations (8%) and observation (10%) (p < 0.001). Diaphragmatic hernia had higher mortality rates than the other pathologies (p < 0.001). Survival rate is increasing to date in newborn pediatric surgery unit; it is independent from parenteral nutrition, maternal age, time to admission and gestational age however it is affected adversely by the age of patient, associating abnormality, low birth weight, presence of sepsis and requirement of respiratory support. Increase in survival could be related to various additional factors such as development of delicate respiratory support machines, broad spectrum antibiotics, hospital infection control teams, central venous catheters, use of TPN by central route, volume adjustable infusion pumps, monitoring devices, neonatal surgical techniques, prenatal diagnosis of pediatric surgical conditions and developments of environmental control methods in neonatal surgical units.The objective was to compare the efficacy and safety of naproxen (NXN) to acetylsalicylic acid (ASA) in the treatment of acute rheumatic fever (ARF). The data of 338 children were retrospectively analyzed. The patients were grouped according to joint and valve involvement and also drug chosen [methyl prednisolone (mPSL), ASA or NXN]. The treatment results and adverse events in each group were compared. The mean age was 10.3 years and the median follow-up was 62 months. Median time for normalization of acute phase reactants was 1 week in patients given steroids and 2 weeks in patients given ASA or NXN. ASA was replaced with NXN in 18 patients (10.2%) due to hepatic toxicity. The rate of rebound, recurrence and the prevalence of rheumatic valve disease were not different in patients given NXN, ASA or mPSL. In conclusion, NXN is a safe and effective alternative to ASA in the treatment of ARF in children.

Pulmonary artery sling and tracheal bronchus in an infant with severe respiratory distress.

A 19-month-old girl was admitted to our clinic with complaints of dyspnea and cough. Her medical history revealed that she had been hospitalized with a diagnosis of bronchiolitis and/or bronchopneumonia for five times during the last year. On physical examination her weight was 8 kg (<3rd percentile), height was 78 cm (10th percentile), she was tachypneic, tachycardic, and she had stridor, suprasternal retractions and coarse crackles over both hemithorax. Echocardiography revealed that the left pulmonary artery (LPA) was originating distal to its normal position, which was consistent with pulmonary artery sling (Fig. 1). Multislice computed tomography demonstrated that the LPA arose from the posterior aspect of the right pulmonary artery and encircled the trachea (Fig. 2). Also, right-upper-lobe bronchus was arising from the right lateral wall of the trachea above the carina (tracheal bronchus, Fig. 3). Because the sling structure was compressing the trachea and causing severe respiratory distress, she underwent left pulmonary arteriopexy operation. The patient’s postoperative course was uneventful and all the respiratory symptoms resolved after the operation. Echocardiography indicated a mild stenosis of LPA which was causing 17 mmHg peak gradient at the level of anastomosis (Fig. 4).

Clinical impact and efficacy of bedside echocardiography on patient management in pediatric intensive care units (PICUs): A prospective study

Objective: To determine the indication and necessity of echocardiographic assessment and therapeutic interventions in critically ill children. Methods: A total of 140 children, including 75 mechanically ventilated (MV) and 65 spontaneously breathing (SB) children, who were admitted consecutively from March to August 2013 were evaluated prospectively. Data regarding the indication for echocardiography and therapeutic approaches used were documented. For evaluating disease severity, the Pediatric Risk of Mortality Score III (PRISM) was ascertained. The correlation between PRISM score and the requirement of echocardiographic evaluations were analyzed. Results: Patients ages were between 45 days to 18 years. The male-to-female ratio was 1.33. In 35.4% patients who underwent echocardiographic evaluation, no definitive alteration occurred in treatment approach, whereas in the remaining 64.6% patients, decisive or supplemental information was gathered. Echocardiography was indicated in 88% MV children and 46.2% SB children. Echocardiographic evaluation was necessary in MV children and there was a positive correlation between the PRISM score and the requirement of echocardiographic assessment (p<0.001). Conclusion: Echocardiographic evaluation is an invaluable tool especially in MV children and the requirement of echocardiographic assessment increases according to clinical severity. Basic training for intensivists in this procedure is crucial and needs to be improved and supported in critically ill.