Transcatheter Closure of Atrial Septal Defect in Children: Single-Center Experience, Mid-Term Follow-up Results.

OBJECTIVE: Atrial septal defect is a congenital heart disease usually diagnosed in childhood. This study aimed to evaluate the mid-term follow-up results of patients who underwent trans- catheter closure of atrial septal defect by comparing the devices and methods used in the procedure and investigating the complications of this procedure in children. MATERIALS AND METHODS: This study evaluated 232 patient files retrospectively. Of the 232 patients, 24 were excluded from the study due to missing files or data. Also, patients with multi-fenestrated atrial septal defect and aneurismatic septal tissue were excluded from the study. The following data were evaluated: follow-up time, patient complaints, symptoms, trans- thoracic echocardiography, and transesophageal echocardiography findings (if performed), the size of the defect as measured by balloon-sizing, the size of the device used in the proce- dure, and major and minor complications. RESULTS: The study included 208 children who were diagnosed with atrial septal defect. The mean age of the patients was 88.0 ± 56.5 months. Of the patients, 170 (81.7%) had no com- plaints. The success rate of the procedure was found to be 95.7%. While device embolization was the most common major complication, arrhythmia was the most common minor complica- tion. The complication rate was statistically different according to the device type used in the procedure. CONCLUSION: Transcatheter closure of atrial septal defect is a safe method for atrial septal defect closure in pediatric patients. The study found that defect diameters measured by differ- ent methods were not correlated with each other. The procedure complication rates differed according to device type.

Discovery of a genetic module essential for assigning left-right asymmetry in humans and ancestral vertebrates.

The vertebrate left-right axis is specified during embryogenesis by a transient organ: the left-right organizer (LRO). Species including fish, amphibians, rodents and humans deploy motile cilia in the LRO to break bilateral symmetry, while reptiles, birds, even-toed mammals and cetaceans are believed to have LROs without motile cilia. We searched for genes whose loss during vertebrate evolution follows this pattern and identified five genes encoding extracellular proteins, including a putative protease with hitherto unknown functions that we named ciliated left-right organizer metallopeptide (CIROP). Here, we show that CIROP is specifically expressed in ciliated LROs. In zebrafish and Xenopus, CIROP is required solely on the left side, downstream of the leftward flow, but upstream of DAND5, the first asymmetrically expressed gene. We further ascertained 21 human patients with loss-of-function CIROP mutations presenting with recessive situs anomalies. Our findings posit the existence of an ancestral genetic module that has twice disappeared during vertebrate evolution but remains essential for distinguishing left from right in humans.

Over 14 years of experience with cardiac myxomas.

Atrial myxomas are the most commonly encountered tumours of the heart and can present at different ages with different clinical symptoms. They are one of the curable tumours of the heart. Appropriate surgical treatment and surgery must be performed with great precautions in order to prevent fatal systemic embolizations. In this retrospective study we will present our experience of 14 years, between 1990 and 2004, in 27 patients who had been operated for cardiac myxomas. Diagnosis of the myxomas were made by echocardiography in all cases. Surgical approach to the tumour was biatrial in nine, left atrial in II, and transseptal in seven patients. Associated procedures included coronary artery bypass grafting in one, mitral valve repair with tricuspid annuloplasty in two patients, mitral valve replacement in one and bilateral femoral embolectomy in one patient. One hospital mortality occurred as a result of multiorgan failure in a patient with peripheral embolization. None of the patients required recurrent operation, however, mitral valve insufficiency was surgically corrected in one patient.

Childhood mediastinal masses in infants and children.

We reviewed all cases of primary pediatric mediastinal masses diagnosed and treated over a 24-year period. In this study, out of 187 primary mediastinal mass cases diagnosed between 1980 and 2004 in Istanbul University Istanbul Faculty of Medicine, Cardiovascular Surgery Department, 37 pediatric primary mediastinal mass cases were retrospectively evaluated according to age, sex, symptoms, diagnostic procedure, anatomical location, surgical treatment, histopathological evaluation and postoperative adjuvant therapy. The patients ranged in age from 2 months to 15 years at the time of diagnosis, with a mean age of 8 years. There were 24 benign (64.8%) and 13 malignant (35.2%) tumors. The cases were lymphoma (27%), neurogenic tumors (21.6%), cystic lesions (18.9%), germ cell tumors (13.5%), thymic lesions (10.8%) and cardiac tumors (8.1%). Complete and partial resections of the tumor were the surgical procedures performed in 24 patients (64.8%) and 3 patients (8.1%), respectively. The three patients with a malignant tumor, in whom the entire mass could not be removed, received chemotherapy and radiation after surgery. In 10 patients with lymphoma, surgery was not a part of treatment and they received medical and radiation therapy after the establishment of the definitive diagnosis. All patients survived and were discharged from the hospital. Except for the cases with lyphoma, all patients are now free of recurrent disease. Compared to adults, children had more lymphomas and neurogenic tumors. Primary pediatric mediastinal malignancies are relatively common in infants and children. Lymphoma, neurogenic tumors and cystic lesions predominated. These differences between the age groups should also be considered when dealing with a mediastinal mass.

Results of surgical treatment for infective endocarditis in children.

OBJECTIVE: Infective endocarditis is uncommon condition, with a high degree of morbidity and mortality. It is less common in children, albeit tending to be associated with congenital cardiac malformations. We describe our experience of the need for surgical treatment in children with infective endocarditis. PATIENTS AND METHODS: We analyzed retrospectively the records of 9 children aged below 16 years seen between May 2003 and March 2005 with infective endocarditis, reviewing the demographic details, clinical presentation, microbiological and echocardiographic data, operative findings, and outcome. RESULTS: Apart from pre-existing renal insufficiency in 1 patient, congenital cardiac malformations were the predisposing factors. Blood cultures were positive in 3, but remained negative in the other 6 patients. The indications for surgical treatment included uncontrolled sepsis, congestive heart failure, recurrent endocarditis, patch or graft dehiscence, and pseudoaneursymal formation. Death due to uncontrolled sepsis resulting in multiorgan failure occurred in 1 patient, who had tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. Another patient died late postoperatively due to cardiac failure after relapse of the endocarditis in the setting of negative blood cultures. CONCLUSION: Despite advances in antimicrobial therapy, diagnosis, and measures of treatment for infective endocarditis, complications continue to be responsible for substantial morbidity and mortality. Since blood cultures are frequently negative, clinical and echocardiographic findings should be the major determinants of strategies used for treatment. We believe that our small series of patients seen over the past two years in which surgical treatment was performed will be helpful in guiding the clinical perspectives for children with infective endocarditis.

Marcadores eletrocardiográficos para detecção precoce de doença cardíaca em pacientes com talassemia beta maior / Electrocardiographic markers for the early detection of cardiac disease in patients with beta-thalassemia major

OBJETIVO: Analisar comparativamente a dispersão da onda P (DOP) em pacientes com talassemia beta maior (β-TM) e indivíduos saudáveis (controles) para a detecção precoce do risco de arritmias. MÉTODOS: Oitenta e uma crianças com β-TM, com idades entre 4 e 19 anos, e 74 crianças saudáveis (grupo controle) foram submetidas a exame eletrocardiográfico e ecocardiograma transtorácico de rotina para avaliação cardíaca. A DOP foi calculada como a diferença entre as durações máxima e mínima da onda P. RESULTADOS: Houve uma diferença estatisticamente significativa entre o grupo de estudo e o grupo controle no pico de velocidade do fluxo transmitral no início da diástole (E) e na razão E/fluxo transmitral tardio (A). A duração máxima da onda P e a DOP foram significativamente maiores nos pacientes com β-TM do que nos indivíduos controles. CONCLUSÕES: O aumento da DOP em nossos pacientes com β-TM pode estar relacionado à depressão na condução intra-atrial, devido à dilatação atrial, e ao aumento da atividade simpática. Estes pacientes devem ser acompanhados atentamente devido à possibilidade de ocorrência de arritmias com risco de vida.

OBJECTIVE: To comparatively evaluate P-wave dispersion (PWD) in patients with β-thalassemia major (TM) and healthy control subjects for the early prediction of arrhythmia risk. METHODS: Eighty-one children with β-TM, aged 4-19 years, and 74 healthy children (control group) underwent routine electrocardiography and transthoracic echocardiography for cardiac evaluation. PWD was calculated as the difference between the maximum and the minimum P-wave duration. RESULTS: There was a statistically significant difference between study and control groups in peak early (E) mitral inflow velocity and E/late (A) velocity ratio. Maximum P-wave duration and PWD were found to be significantly higher in β-TM patients than in control subjects. CONCLUSIONS: Increased PWD in our β-TM patients might be related to depression of intra-atrial conduction due to atrial dilatation and increased sympathetic activity. These patients should be closely followed up for risk of life-threatening arrhythmias.