Usefulness of Kawasaki disease risk scoring systems to the Turkish population

Objective: Kawasaki disease (KD) is the most common cause of coronary artery aneurysm (CAA) in children. The available risk scores to predict intravenous immunoglobulin (IVIG) resistance and CAA were developed in Asian populations in whom their effectiveness has been proven, but data on non-Asian children are limited. This study aimed to evaluate the ability of 5 risk scoring systems to predict IVIG resistance and CAA in Turkey patients with KD

NATURAL HISTORY OF CONGENITAL ISOLATED MILD AORTIC VALVE AND MILD PULMONARY VALVE STENOSIS: A SINGLE-CENTER FOLLOW-UP STUDY

Objective: Most of the available information on the natural history of aortic stenosis (AS) and pulmonary stenosis (PS) in children is based on studies carried out over the past 35-40 years using cardiac catheterization. This study aimed to reveal the natural history of congenital isolated mild valvular AS and PS in children using serial Doppler echocardiographic examinations

Clinical assessment and echocardiography follow-up results of the children with acute rheumatic fever

Acute rheumatic fever (ARF) is an inflammatory collagenous tissue disease which shows its cardinal signs in joints, heart, skin and nervous system while affecting whole connective tissue system more or less. This study was conducted in order to investigate the clinical pattern and severity of ARF, echocardiographic findings and the course of the patients with heart valve involvement by studying the clinical and laboratory aspects of the patients diagnosed with ARF according to updated Jones criteria. The study included 214 patients diagnosed with ARF for the first time between January 2005 and May 2008. All patients were scanned with doppler echocardiography (ECHO) between certain intervals. Severity of carditis was grouped into 3 groups of mild, moderate and severe. The frequency of carditis was 57.9%, arthritis was 73.4%, chorea was 11.7% and erythema marginatum was 0.9% but no subcutaneous nodules. Recovery was observed in 22% of the cases of isolated aortic insufficiency (AI), 50% of the cases with isolated mitral insufficiency (MI) and 80% of the cases with mitral and aortic insufficiencies together (MI+AI). Recovery in isolated MI was significantly much more than recovery in isolated AI. However, recovery in AI was significantly much more than in MI in cases of mitral and aortic insufficiencies together. In conclusion, ARF is a cause of acquired and preventable heart disease and it can be reversed through right diagnosis and appropriate treatment. Isolated mitral insufficiency, isolated aortic insufficiency and both mitral and aortic insufficiency are observed during a valvular disease. Remission among valvular diseases are most commonly in those with mitral insufficiency and remissions in both mitral and aortic insufficiency occur most commonly in aortic ones. Regular prophylaxis is the key element for long term prevention of patients with ARF.</p

An incidentally detected anomalous origin of the right coronary artery from the pulmonary artery in an infant

Isolated anomalous origin of the right coronary artery from the main pulmonary artery is a rare congenital anomaly, and few cases have been reported in the pediatric age group. Here in, we report an asymptomatic case of a 2-month-old male infant who has been diagnosed as anomalous origin of the right coronary artery from the main pulmonary artery during the evaluation for cardiac abnormalities. For a suspicion on echocardiography, cardiac catheterization and coronary angiography performed to verify the diagnosis of anomalous origin of the right coronary artery from the main pulmonary artery. The patient underwent surgery and did well after two months follow up. Early diagnosis may prevent patients from cardiovascular complications

Transient complete atrioventricular block in a patient with Blackfan-Diamond anemia due to severe hypothyroidism

We report a 14-year-old boy with a previous diagnosis of Blackfan-Diamond anemia (BDA) who presented with the complaint of bradycardia. Electrocardiogram (ECG) showed complete atrioventricular (AV) block with the mean heart rate 51 bpm on admission. Laboratory data revealed endocrine abnormalities and severe hypothyroidism. Soon after chelation and levothyroxine therapy, AV block was disappeared gradually

Right Atrial Appendage Aneurysm in a Newborn Diagnosed with Fetal Echocardiography

Right atrial appendage aneurysm is a very rare condition which can be asymptomatic or can cause arrhythmia or life-threatening thromboembolism. We report a case of newborn with right atrial appendage aneurysm who was diagnosed with fetal echocardiography. Anticoagulant therapy was applied to prevent thromboembolism and he is still going on follow-up without any complaint

Multidisciplinary approach to congenital multiple arterio-porto-caval malformation: Case report

A case of intrahepatic arteriovenous malformation connected with umbilical, hepatic, portal, and arterial systems in a fetus diagnosed at 36 weeks of gestation is presented. Prenatal color Doppler ultrasonography in the fetal liver demonstrated complicated vascular connections fed by arterial and portal branches. Postnatal color Doppler, power Doppler, 3D power Doppler, abdominal multidetector computed tomography (MDCT), and angiography CT verified the prenatal diagnoses. Extended right hepatectomy was performed successfully on postnatal day 19

Detection of Early Right Ventricular Dysfunction in Young Patients With Thalassemia Major Using Tissue Doppler Imaging

Background Myocardial iron overload is the most common cause of mortality in patients with thalassemia major (TM), also known as beta-thalassemia. T2* cardiovascular magnetic resonance imaging (MRI) is the best way of monitoring cardiac iron, and new echocardiographic techniques can be used to assess cardiac function. Objectives The aim of this study was to assess the systolic and diastolic right ventricular (RV) function of patients with TM using tissue Doppler imaging (TDI) and to determine whether this echocardiographic technique is an adequate diagnostic tool for the screening and detection of subclinical cardiac dysfunction. Patients and Methods Eighty-four patients with TM were evaluated by conventional echocardiography and pulse-wave TDI. The data of the TM group (Group 1) were compared with that of 85 age- and sex-matched healthy controls (Group 2). Cardiovascular T2* MRI examinations were performed in 49 of the 85 patients. Results The patients with TM had significantly lower values for weight, height, body mass index, systolic arterial pressure, deceleration time, E’/A’, and ejection time (ET) than the controls. Group 1 also had significantly higher values for peak early diastolic velocity (E) over peak late diastolic velocity (A), peak early diastolic velocity of TDI (E’), peak late diastolic velocity of TDI (A’), E/E’, isovolumetric relaxation time, isovolumetric contraction time, and RV magnetic perfusion imaging (MPI) than Group 2. Conclusions RV diastolic dysfunction occurs before systolic deterioration in patients with TM and cannot be screened with conventional echocardiographic techniques. In routine practice, TDI measurements, MPI (for global function) and the E/E’ parameter (for diastolic function) can be used to screen and detect early RV dysfunction

Regression of Cardiac Rhabdomyomas in a Neonate after Everolimus Treatment

Cardiac rhabdomyoma often shows spontaneous regression and usually requires only close follow-up. However, patients with symptomatic inoperable rhabdomyomas may be candidates for everolimus treatment. Our patient had multiple inoperable cardiac rhabdomyomas causing serious left ventricle outflow-tract obstruction that showed a dramatic reduction in the size after everolimus therapy, a mammalian target of rapamycin (mTOR) inhibitor. After discontinuation of therapy, an increase in the diameter of masses occurred and everolimus was restarted. After 6 months of treatment, rhabdomyomas decreased in size and therapy was stopped. In conclusion, everolimus could be a possible novel therapy for neonates with clinically significant rhabdomyomas