Nazan Özbarlas

Closure of patent ductus arteriosus in children, small infants, and premature babies with Amplatzer duct occluder II additional sizes: Multicenter study

To evaluate safety and efficacy of closure of patent ductus arteriosus (PDA) with Amplatzer duct occluder II Additional Sizes (ADO II AS) and to report early and midterm results of the device in children and very young symptomatic infants.

Marked Bradycardia Due to Pulsed and Oral Methylprednisolone Therapy in a Patient with Rapidly Progressive Glomerulonephritis

Accessible online at: http://BioMedNet.com/karger Dear Sir, Pulsed methylprednisolone (MP) is becoming very popular in the treatment of various immunomediated disorders, especially in the field of pediatric nephrology. Although associations with serious cardiac arrhythmias and even sudden death have been reported in sporadic cases [1, 2], its exact mechanism of action is not clear. We were also not able to find any case report stating oral MP as a possible cause of cardiac side effects. Herein we report a patient with rapidly progressive glomerulonephritis who developed marked sinus bradycardia necessitating cardiac pacing after a first dose of pulsed MP and showed continuity of sinus bradycardia with oral MP regimen. A 14-year-old boy was admitted to our hospital with hematuria and edema. Physical examination revealed blood pressure of 140/90 mm Hg and a regular pulse of 104 b.p.m. He had mild pretibial edema, the other findings including the cardiovascular system were all normal. Laboratory examination revealed; hemoglobin 11 g/dl, normal leukocyte and platelet counts; blood urea nitrogen 105 mg/dl, serum creatinine 5.2 mg/ dl, serum uric acid 9.2 mg/dl and serum albumin 4.2 g/dl, erythrocyte sedimentation rate 130 mm/h, serum electrolytes and calcium were normal. Creatinine clearance was 17.2 ml/min/1.73 m2. Urinary findings were hematuria, protein 4+, large erythrocytes, 10–15 leukocytes and occasional hyaline cast in the sediment. Quantitative proteinuria was 341 mg/m2/h. An abdominal sonogram revealed normally sized kidneys. Echocardiographic findings were normal. Serological tests showed a negative antinuclear antibody, negative anti-DNA antibody and normal C3 level. Chest X-ray and electrocardiogram were normal. Clinical diagnosis was rapidly progressive glomerulonephritis, and he was given a dose of 30 mg/kg pulsed intravenous MP. Two other consecutive daily pulses were planned. Five hours later, heart rate decreased to 50 b.p.m. and an ECG obtained at this time showed sinus bradycardia with no heart block or arrest. Since the heart rate had decreased below 40 b.p.m. a transvenous temporary pacemaker was positioned and pulsed MP therapy was discontinued. His heart rate gradually increased to 80 b.p.m. in 3 days. He was followed up for another 3 days with normal heart rate, then he was given oral MP 60 mg/m2 daily. Surprisingly his heart rate decreased again gradually and reached 40 b.p.m. in 5 days. This marked sinus bradycardia forced us to discontinue oral MP therapy on the 8th day of treatment. A percutaneous renal biopsy was performed and the diagnosis of membranoproliferative glomerulonephritis was established. He was started on oral cyclophosphamide and dipyridamole treatment. After cessation of oral MP, his heart rate increased to normal levels. Intravenous steroids are frequently used in the treatment regimen of all types of glomerulonephritis, vasculitis and collagen tissue disorders [3, 4]. Although the side effects of steroids are all well known, there are only few reports in the literature of side effects on the cardiovascular system. The half-life of intravenous MP is 3 h and the duration of the effect on the hypothalamic-pituitary-adrenal axis is 12–36 h after a single dose. In our case, sinus bradycardia was seen 5 h after infusion. After 3 days, the patient’s heart rate was normal, but following oral MP treatment the heart rate decreased again. This observation indicates that oral MP treatment could be the cause of decreased heart rate. Premature ventricular contractions, atrial fibrillation, myocardial infarction, junctional arrhythmias, asystole and sudden death were reported with pulsed intravenous MP treatment [5, 6]. The mechanism is not obvious, but it was suggested that MP may have a direct effect on the integrity of cardiac or smooth muscles, causing alterations in calcium and potassium flux across cell membranes. The speed of administration has been suggested as an important factor [7]. Our experience suggests that both intravenous and oral MP treatments may cause adverse effects on the cardiovascular system. We recommend that patients receiving highdose MP should be carefully monitored for cardiac side effects.

Prevalence and distribution of structural heart diseases in high and low risk pregnancies

OBJECTIVEnTo establish and compare the frequency of intrauterine congenital heart defects in high-risk and low-risk pregnancies for congenital heart diseases.nnnMETHODSnRecords of 3782 patients who underwent fetal echocardiography at the Pediatric Cardiology Unit were reviewed for reasons of referral and results of echocardiography retrospectively. The categorical variables between the groups were analyzed using a Chi-square test.nnnRESULTSnStructural heart defects were found in 213 (5.6%) fetuses in both high and low risk groups. Most common defects were ventricular septal defect (36.2%) and atrioventricular septal defect (14.1%). Frequency of congenital heart diseases was 7.8% (169 fetuses) in high-risk group: 4.1% were complex, 2.3% significant and 1.4% were minor. In low-risk group, the frequency was 2.7% (44 fetuses): 0.6% were complex, 0.8% significant and 1.3% were minor. In this group, rates of congenital defects were high regarding particular reasons: intrauterine fetal death in previous pregnancy (6.3%), abnormal first or second trimester screening tests (4.3%), and multiple gestations (3.4%). The sensitivity and specificity of fetal echocardiography according to transthoracic echocardiography were found to be 86% and 99%, respectively.nnnCONCLUSIONnCongenital heart diseases rate, as expected, was found to be higher in high-risk group. However, regarding intrauterine fetal death in previous pregnancy, abnormal first or second trimester screening tests and multiple gestation, no statistically significant difference was observed between low-risk and high-risk groups. Therefore, we suggest the routine use of fetal echocardiography in cases we have mentioned above if the staff and equipment of the pediatric cardiology clinic are eligible.

Mitral annular calcification and brown tumor of the rib in a child with chronic renal failure

Mitral annular calcification and liquefaction necrosis of this lesion mimicking intracardiac tumor because of secondary hyperparathyriodism have been described in adult patients with chronic renal failure, but have not been reported in children. Chronic renal failure is one of the predisposing factors of this condition. We report the case of a 13-year-old patient with continuous ambulatory peritoneal dialysis with severe hyperparathyroidism who was found to have intracardiac and rib lesions considered to be brown tumors.

[Long term follow-up of 799 children with isolated ventricular septal defects].

OBJECTIVESnWe evaluated long-term follow-up results and prognosis of pediatric patients with isolated ventricular septal defects (VSD).nnnSTUDY DESIGNnThe 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).nnnRESULTSnThe 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.nnnCONCLUSIONnOur 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.

Systemic pulmonary shunt performed with Shelhigh internal mammary artery: early results.

OBJECTIVEnThe purpose of this study was to evaluate early findings for aortopulmonary shunts using bovine internal mammary artery grafting.nnnMETHODSnBovine internal mammary artery grafts biomodified with glutaraldehyde and the Shelhigh No-React process were used between May 2005 and April 2006 in our clinic for 20 cases of aorta-pulmonary artery shunts. We implanted 2 sizes of grafts, either a graft with 4-mm proximal and 5-mm distal diameters or a graft with 5-mm proximal and 6-mm distal diameters. Patients were between 20 days and 7 years of age, and the sex distribution was 55% female and 45% male. A Blalock-Taussig shunt with left thoracotomy was performed in patients 2 years of age and older, and a central shunt with sternotomy was performed for patients younger than 2 years. Eight patients underwent operation under emergency conditions. Nine patients had tetralogy of Fallot and/or pulmonary atresia (PA); 3 had transposition of the great arteries, ventricular septal defect, and pulmonary stenosis (PS); 3 had tricuspid atresia; 3 had PS and double-inlet left ventricle; and 2 had PA.nnnRESULTSnAfter the operation, immediate recovery of oxygen saturation and partial oxygen pressure was observed in all patients. Pulse oximetry measurements showed patient oxygen saturation to be between 84% and 100%. One patient underwent reoperation at the third postoperative hour because of bleeding. Two patients died from causes unrelated to the graft. The hospitalization period was between 7 and 29 days. Echocardiography evaluations showed no shunt obstruction for the early (first postoperative week) or middle (postoperative week 24) period.nnnCONCLUSIONnWith this study, we assessed the use of the Shelhigh internal mammary artery graft instead of synthetic (polytetrafluoroethylene) tubular grafts in shunt operations for congenital heart diseases with decreased pulmonary blood flow and no evidence of calcification. Further investigation involving a larger number of cases and longer-term results for patency are needed to confirm our findings.

Congenital aorto-left atrial tunnel--an unusual communication: a case report.

Congenital aortico-cameral communications are rarely seen. We present an asymptomatic patient in whom there was a congenital vascular communication rising from the aortic root and terminating in the left atrium. She had an atrial septal defect (ASD). The diagnosis was made with echocardiography and confirmed by aortography. She was treated by closing the tunnel and the ASD. The outcome was satisfactory in this extremely rare case of a congenital cardiac lesion. Coincidental diagnosis could be made during careful echocardiographic examination.

Delivering stents in congenital heart disease using the double‐wire technique: Technical considerations

To evaluate the use of a double‐wire technique to deliver stents.

'Natural Conduit' between two atria associated with atrial septal defect.

Herein, we present a case of 46 years old woman with an extracardiac venous connection between two atria associated with secundum type atrial septal defect (ASD). This interatrial tunnel was not recognized by both transthoracic and transesophageal echocardiographic examinations. Computed tomography suspected a blood flow from right inferior pulmonary vein to inferior caval vein. Consequently, the patient underwent cardiac catheterization. Angiography revealed a tunnel between left and right atrium. To our knowledge, this is the first report of a case with venous tunnel between two atria associated with secundum type ASD in the literature.

Troponin T and NT ProBNP Levels in Gestational, Type 1 and Type 2 Diabetic Mothers and Macrosomic Infants.

This study compares NT proBNP and troponin T levels in umbilical cord arterial blood and postnatal echocardiographic findings for infants of gestational and pregestational diabetic mothers and macrosomic infants. Twenty-seven infants of pregestational diabetic mothers, 61 infants of gestational diabetic mothers and 37 macrosomic infants of nondiabetic mothers were prospectively enrolled in this study along with a control group of 58 healthy infants of mothers without any pregestational or gestational disorders as the control group. All enrollees were born after 34xa0weeks of gestation. For this study, umbilical cord blood was drawn during delivery to determine NT proBNP and troponin T levels. Echocardiography was performed 24–72xa0h after the delivery. Umbilical cord troponin T and NT proBNP levels were found to be higher in the diabetic and macrosomic groups than in the control group (all of them pxa0<xa00.001). NT proBNP levels were positively correlated with interventricular septum thickness in the pregestational and gestational infants of diabetic mothers groups (rxa0=xa00.564 and rxa0=xa00.560, respectively, pxa0<xa00.01). Both pregestational and gestational diabetic mothers were divided into two groups according to HbA1c levels in the third trimester as good (<6.1xa0%) and suboptimal (>6.1xa0%) metabolic control. In the good and suboptimal metabolic control diabetic groups, NT proBNP levels were also positively correlated with interventricular septum thickness (rxa0=xa00.536 and rxa0=xa00.576, respectively, pxa0<xa00.01). In the suboptimal metabolic control diabetic group, NT proBNP was only found to be positively correlated with the left ventricular mass index (rxa0=xa00.586, pxa0<xa00.01). While there was no correlation in the myocardial performance index between infants of diabetic mothers and the control group, the myocardial performance index of macrosomic infants was lower than that of the control group (pxa0=xa00.017). Cardiac biomarkers (NT proBNP and troponin T) were elevated in infants of diabetic mothers and macrosomic infants. While there was a positive correlation between NT proBNP levels and cardiac structure in infants of pregestational and gestational diabetic mothers, there was no relationship between NT proBNP levels and cardiac function.