Ümrah Aydoğan

Congenital heart disease in children with Down's syndrome: Turkish experience of 13 years.

Background — Down’s syndrome (DS) is the most common chromosomal abnormality due to a trisomy of chromosome 21 commonly associated with congenital heart defects (CHDs). This study aimed to evaluate the frequency and types of CHD patterns in Turkish children with DS. Method — The data relate to paediatric patients with DS who underwent cardiologic screening between 1994 and 2007 and were reviewed in our Paediatric Cardiology unit. Results — Four hundred and twenty-one out of the 1042 paediatric patients with DS studied over a 13-year period had associated CHD. Of these, 320 (77.6%) had a single cardiac lesion, while the remaining 92 patients (22.4%) had multiple defects. The most common single defect was an atrioventricular septal defect (AVSD) found in 141 patients (34.2%), followed by 69 patients (16.7%) showing secundum type atrial septal defect, and ventricular septal defect in 68 patients (16.5%). AVSDs were the leading type, isolated or combined with other cardiac anomalies with an overall occurrence of 19.8% of paediatric patients with DS, and 49.2% of paediatric patients with both DS and CHD. Conclusion — This is the first study concerning the frequency and type of CHD observed in Turkish children with DS.The high frequency of AVSD in Turkish children with DS implied that early screening for CHDs by echocardiography is crucial. The correction of AVSDs in paediatric patients with DS should be performed in the first 6 months of life to avoid irreversible haemodynamic consequences of the defect.

Transcatheter embolization treatment of coronary arteriovenous fistulas.

Transcatheter coronary arteriovenous fistula occlusion was performed in 5 children with a median age of 5.3 years (range, 0.7 to 11 years). Surgical ligation had been carried out previously in one case, but recanalization occurred during follow-up. Dilation of the feeding artery was observed in 4 patients, with an additional aneurysm of the coronary artery in one of these. Occlusion was attempted three times in 1 patient, twice in 2, and once in the other 2. Detachable balloons were used in 2 procedures, and controlled-release coils in 3. Complete occlusion of the fistula was achieved in 4 patients. In the 5th patient, occlusion of one of two fistulas was unsuccessful, and mild to moderate aortic valve insufficiency developed due to catheter manipulation. Our experience indicates that transcatheter occlusion of congenital coronary arteriovenous fistula is at least as successful as surgical intervention. Multiple occlusion procedures can be avoided by selecting the optimal technique in each case.

Transcatheter closure of a fistula between the right pulmonary artery and left atrium using the Amplatzer septal occluder

A congenital fistula between the right pulmonary artery (RPA) and left atrium (LA) is a rare condition that results in central cyanosis. An 11-year-old boy was admitted with exertional dyspnea and easy fatigability. He had severe cyanosis of the lips and limbs with clubbing of the fingers. Systemic oxygen saturation was 70%. There was no abnormal finding on electrocardiography, chest radiography, and echocardiography. Agitated saline injection showed early appearance of contrast bubbles in the LA. A pulmonary arteriovenous fistula was suspected and diagnostic cardiac catheterization was performed. Angiography demonstrated a large fistula between the proximal RPA and LA. The narrowest part of the fistula was 13.8 mm in balloon sizing. A 14-mm Amplatzer septal occluder was deployed at the narrowest site; however, the device migrated to the LA and then to the aortic arch. The device was removed and was successfully reimplanted to the fistula. After the procedure, arterial oxygen saturation increased from 70% to 96% and control angiography demonstrated complete occlusion of the fistula. The patient was symptom-free on follow-up evaluations at 6, 12, and 18 months, with a mean oxygen saturation of 96%. This case represents the first pediatric patient in whom a septal occluder was used.

Electrocardiographic markers for the early detection of cardiac disease in patients with beta-thalassemia major

OBJECTIVE To comparatively evaluate P-wave dispersion (PWD) in patients with beta-thalassemia major (TM) and healthy control subjects for the early prediction of arrhythmia risk. METHODS Eighty-one children with beta-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 beta-TM patients than in control subjects. CONCLUSIONS Increased PWD in our beta-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.

Arterial duct closure with detachable coils: application in the small child.

Transcatheter closure of patent ductus arteriosus using controlled-release coils was performed in 16 patients weighing < 10 kg. No embolization occurred. Procedure-related complications occurred in 3 patients (18.8%): massive femoral hemorrhage in 1 and femoral artery thrombosis in 2. The ductus recanalized in 1 of them because of mechanical hemolysis caused by streptokinase treatment. This was the only patient who underwent another occlusion procedure. Complete occlusion was achieved in 7 patients (43.8%) immediately, in 13 (81.2%) the following day, and in all 15 patients who had completed the 6-month follow-up. During follow-up, flow velocities between the left and the main pulmonary arteries and between the descending and the ascending aortae did not differ significantly. Flow velocity was > 2 m·sec−1 in 3 patients in the left pulmonary artery and in 1 in the descending aorta. Protrusion of the coil was seen in 3 of these patients. Flow velocity was also high in the main pulmonary artery in the 4th patient. In conclusion, coil occlusion of ductus arteriosus is feasible in the small child, but no more than half a loop of the coil should be left at the pulmonary site. High flow velocity does not always mean obstruction.

The effects of surgical repair on P-wave dispersion in children with secundum atrial septal defect

IntroductionAtrial septal defect (ASD) is one of the most common congenital heart diseases in children. P-wave dispersion has been reported to be associated with non-homogeneous propagation of sinus impulses. The heterogeneity of atrial conduction time may predispose the atria to arrhythmias. The aim of this study was to determine the impact of surgical repair on P-wave indices in children with isolated secundum ASD.MethodsChildren with isolated secundum ASD undergoing surgical repair (n=50; mean age, 7.0±3.0 years) and healthy controls (n=51; mean age, 7.6±2.7 years) were compared. Maximum P-wave duration (Pmax), shortest duration (Pmin) and P-wave dispersion (Pd) were measured using 12-lead surface electrocardiography.ResultsMean Pmax was found to be significantly higher in children with ASD compared with controls (95.2±10.8 vs 84.1±9.2 msec; P<0.001), and Pd before surgery was significantly higher compared with controls (47.4±12.0 vs 38.8±9.7 msec; P<0.001). Both P-wave indices were significantly decreased within the first year after surgical closure — the values decreased to those comparable to healthy controls (Pmax, 86.2±9.7 msec; Pd, 39.8±10.7 msec; P>0.05).ConclusionSurgical closure of ASD in children decreases Pmax and P-wave conduction time. We speculate that earlier closure of the defect may play an important role in avoiding permanent changes in the atrial myocardium and atrial fibrillation in adulthood.

Electrocardiographic and echocardiographic findings in street children known to be substance abusers

Substance abuse is prevalent in adolescent street children, and death is reported as secondary to aspiration, accidental trauma, asphyxia, cardiac arrhythmia, anoxia, vagal inhibition and respiratory depression. In this study, we examined electrocardiographic and echocardiographic findings from 53 street male adolescents, comparing our findings to those obtained from 61 controls in the same age group. The street children smoked cigarettes (98.1%) and had used, or were using, thinner (73.6%), glue (75.5%), hashish (79.2%), morphine or its products (24.5%), ecstasy (37.7%), anti-emetics (13.2%) and alcohol (60.4%). On examination, their blood pressures were lower than the control group. Electrocardiographically, PR, QRS, QT were found to be longer (p less than 0.05) than the values for healthy controls. Although it was not statistically significant, QTc duration was also longer than the control group. Echocardiography revealed increased diameters of the left ventricle and atrium, the aorta, and the coronary arteries as compared to the healthy children (p less than 0.05).

[The comparison a 16-year follow-up results of balloon angioplasty for aortic coarctation in children of different age groups: a single-center experience].

OBJECTIVE Pediatric patients with different age groups who underwent balloon angioplasty for aortic coarctation were evaluated for recoarctation, aneurysm, peripheral arterial injuries and concomitant diseases. METHODS From January 1994 to 2010, 80 patients with aortic coarctation (native/recoarctation) were evaluated, retrospectively. According to age at angioplasty, patients were divided into three groups: Group A (0-3 months, n=29, 25 male/4 female, average weight 4±1.2 kg), Group B (3-12 months, n=20, 15 male/5 girls, average weight 6.5±1.9 kg) and Group C (> 1 year, n= 31, 15 male/16 girls, average weight 22.8±16 kg). The patients were followed with echocardiography and clinical signs. The data of the native and recoarcted patients and also those in three different age groups were analyzed by using Chi-square, Kruskal-Wallis, and Student t tests. RESULTS Peak systolic pressure gradient was reduced from 42±17 mmHg to 6.2±6 mmHg after balloon angioplasty (p<0.001) [n=80, 56 (70%) native, 24 (30%) recoarctation]. There was no difference between groups for early success. None of them did require immediate surgery. There were ventricular septal defect in 23 (28.7%), bicuspid aorta in 18 (22.5%), patent ductus arteriosus in 11 (13.7%) patients. Two patients had Turners syndrome. Mean follow-up period was 74±56 months. While recoarctation developed in 20 (25%), [12 (60%) in Group A, 5 (25%) in Group B and 3 (15%) in group C, incidence was higher in the 0-3 months age group (p=0.018). Femoral artery occlusion and aneurysm were developed in 6 (7.5%) and 4 (5%) patients, respectively, and all of them were under 1-year-old. CONCLUSION Balloon angioplasty can be used a method in treatment of native aortic coarctation and postoperative restenosis. Especially, children under 3 months should be monitored closely after the procedure for recoarctation, aneurysms and peripheral artery problems.

[A rare cause of recurrent wheezing and respiratory distress: Scimitar syndrome].

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.

Left-sided aortic arch, right-sided descending aorta, and right-sided arterial duct associated with interruption of the aortic arch and presence of Abbott’s artery

We describe a male infant, seen at the age of 10 days, with a very rare form of vascular ring. The aortic arch was left-sided, but the aorta descended on the right, with a right-sided persistently patent arterial duct associated with interruption of the aortic arch and presence of Abbotts artery. We performed end-to-side anastomosis of the descending aorta to the ascending aorta, divided the Abbotts artery and the right-sided arterial duct, and banded the pulmonary trunk. Despite our best efforts, the patient died on the eighth day after the surgical intervention.