Alper Guzeltas

Echocardiographic Follow-Up of Children with Isolated Discrete Subaortic Stenosis

This study evaluates the progression of stenosis, onset and progression of aortic regurgitation (AR), and the results of surgical outcomes in children with isolated discrete subaortic stenosis (SAS). The medical records of 108 patients (mean age, 5.5 ± 3.8 years; range, 3 days to 18 years) with isolated discrete SAS were reviewed. Patients with lesions other than AR were excluded. Very mild stenosis was defined as Doppler peak systolic instantaneous gradient (PSIG) less than 25 mmHg, mild stenosis as 25–49 mmHg, moderate stenosis as 50–75 mmHg, and severe stenosis as more than 75 mmHg. Seventy-eight of 108 patients were followed for 2 months to 14 years (mean, 4.8 ± 3.7 years; median, 5 years) with medical treatment alone. In these patients, the mean PSIG at last echocardiogram was higher than the mean PSIG at initial echocardiogram (39 ± 19 vs 31 ± 12 mmHg, respectively; p < 0.001). Among 24 patients with very mild stenosis at initial echocardiogram, 10 had mild and 2 had moderate stenosis after a mean period of 5.6 years. Among 46 patients with mild stenosis at initial echocardiogram, 11 had moderate and 5 had severe stenosis after a mean period of 4.1 years. Only 1 patient among the 8 patients with moderate stenosis at initial echocardiogram had severe stenosis after a mean period of 2.7 years. Thirty-nine patients (50%) had AR (13% trivial, 33% mild, and 4% moderate) at initial echocardiogram. After a mean period of 4.8 years, 77% of the patients had AR (10% trivial, 53% mild, 9% mild–moderate, and 5% moderate). Twenty-four patients underwent surgery. Preoperatively, mean Doppler PSIG and AR incidence were 64 ± 17 mmHg and 91% (22/24), respectively. The mean Doppler PSIG was 30 ± 19 mmHg and AR was present in all of the patients a mean period of 4.1 years after surgery. Two patients underwent reoperation for recurrent SAS and AR. Patients with very mild or mild stenosis may be followed noninvasively every year. One patient of the 8 patients with moderate stenosis progressed to severe stenosis, and moderate AR developed in 2 patients after a mean of 2.7 years. We recommend that patients with moderate stenosis undergo careful evaluation to determine whether surgery is necessary due to the severity of stenosis and AR.

Stent implantation in the arterial duct of the newborn with duct-dependent pulmonary circulation: single centre experience from Turkey

OBJECTIVES Implantation of stents into the ductus arteriosus is an alternative treatment to palliative or corrective cardiac surgery in newborns with duct-dependent pulmonary circulation, although the use of this technique for congenital heart disease is limited. METHODS Between April 2010 and June 2011, 13 patients underwent patent ductus arteriosus stenting after full assessment by echocardiogram and angiogram, two of patients had pulmonary atresia (PA) and ventricular septal defect (VSD), six patients had PA with intact ventricular septum (IVS), four patients had critical pulmonary stenosis with IVS and one single ventricle physiology with PA and four patients had radiofrequency-assisted perforation of the pulmonary valve at the same time. All procedures were retrograde through the femoral artery, except one, which was by the femoral vein approach. RESULTS The mean age and weight during intervention were 10.5±5.7 days and 3.1±0.4 kg, respectively. The mean of procedure and scopy time, time of stay in intensive care, total out-of-hospital and total follow-up time were 138.88±67.11 min; 40.32±25.86 min; 4.88±6.07 days; 11.00±6.89 days and 86.40±73.21 days, respectively. The mean of the radiation amount was 1054.27±1106.91 cGy/cm2. The mean of saturation before and after intervention were 64.44±5.83; 81.88±6.95%, respectively. Procedure-related deaths were observed in two patients. The causes of death were pulmonary haemorrhage (n=1) and retroperitoneal haemorrhage (n=1). Two patients also died after discharge before surgery due to sepsis (n=1) and aspiration pneumonia (n=1). Eight of 13 patients achieved stent patency during 6 months of follow up and re-stenosis developed in one patient (1/8; 12.5%) who had undergone a Glenn operation at 4.5 months of age. CONCLUSIONS Ductal stenting is a practicable, effective, safer and less invasive method compared palliative or corrective surgery. Patients with ductal stenting have growth of the pulmonary artery which provides additional time for surgical repair. Our data suggested that ductal stenting should be considered as a first treatment step in newborns with duct-depended pulmonary circulation. However, long-term palliation without stent re-stenosismight still be a concern especially in patients with hypoplastic pulmonary arteries.

Evaluation of Permanent or Transient Complete Heart Block after Open Heart Surgery for Congenital Heart Disease.

The features of pediatric patients with postoperative transient and permanent complete atrioventricular (AV) block (CAVB) were compared.

Percutaneous Pulmonary Valve Implantation Using Edwards SAPIEN Transcatheter Heart Valve in Different Types of Conduits: Initial Results of a Single Center Experience

BACKGROUND Percutaneous pulmonary valve implantation is frequently used as a less invasive method in patients with conduit dysfunction. The common valve type cannot be used in conduits with a diameter larger than 22 mm. There has been limited experience concerning the used of the SAPIEN Transcatheter Heart Valve, produced for use in conduits with a large diameter. This study presents hemodynamic and early follow-up results from a single center in Turkey concerning the use of the SAPIEN Transcatheter Heart Valve in different types of conduits and different lesions. PATIENTS AND METHOD Between October 2010 and July 2012, seven SAPIEN Transcatheter Heart Valve implantations were performed. There was mixed type 2 pure insufficiency with stenosis and insufficiency in five patients. Three different conduits were used, and one native pulmonary artery process was performed. Patients were followed for hemodynamic findings, functional capacities, valve competence, reshrinking, and breakage in the stent, and the results were evaluated. RESULTS Implantations were successfully performed in all patients. Right ventricular pressures and gradients were significantly reduced, and there was no pulmonary regurgitation in any patient. Functional capacities evidently improved in all patients except for one with pulmonary hypertension. No major complication was observed. During the mean time of follow-up (7.2 ± 4.7 months), no valve insufficiency or stent breakage was observed. CONCLUSION Procedural results and short-term outcomes of the SAPIEN Transcatheter Heart Valve were very promising in the patients included in the study. The SAPIEN Transcatheter Heart Valve can be a good alternative to surgical conduit replacement, particularly in patients with larger and different types of conduits.

Electrocardiographic and electrophysiologic effects of dexmedetomidine on children.

Dexmedetomidine (DEX) is a highly selective alpha‐2‐adrenergic agonist approved for short‐term sedation and monitored anesthesia care in adults. Its effects on the electrocardiography and cardiac conduction tissue are not well described in the literature. Therefore, we aimed to characterize the electrocardiographic and electrophysiologic effects of DEX in children.

Evaluation of non‐surgical causes of cardiac tamponade in children at a cardiac surgery center

The aim of this study was to examine the causes of cardiac tamponade in children undergoing percutaneous pericardiocentesis.

Dispersion of the P wave as a test for cardiac autonomic function in diabetic children.

OBJECTIVE We aimed, in this study, to compare dispersion of the p wave in patients with type 1 diabetes to nondiabetic control subjects, and to investigate the relationship between the dispersion of the p wave and cardiac autonomic dysfunction in diabetic children. METHODS We enrolled 49 patients with type 1 diabetes, and 32 age- and sex-matched healthy subjects, measuring the Valsalva ratio, resting heart rate, and orthostatic hypotension in all. The duration of the p wave was measured manually on a high-resolution computer screen. Dispersion, defined as the difference between maximum and minimum durations of the p waves, was also measured in the 12-lead electrocardiogram before and after the Valsalva maneuver. RESULTS The mean age of the patients and their controls were 14.2 +/- 4.8 years, and 12.7 +/- 4.5 years, respectively. The mean duration of diabetes had been 6.2 +/- 4.6 years. Maximal and minimal values for the duration of the p wave were significantly decreased in the diabetic children, with the dispersion itself significantly increased. Values for the dispersion in the diabetic subjects were similar before and after the Valsalva maneuver, whereas dispersion was found significantly increased after this maneuver in the controls. The differences in the Valsalva ratio, resting heart rate, and orthostatic hypotension between the groups, on the other hand, were not found to be statistically significant. CONCLUSION The noted increase in the dispersion of the p wave in diabetic children reveals the onset of cardiac electrophysiological heterogeneity before it is possible to detect parasympathetic and sympathetic dysfunction with other tests.

Outcome for patients with isolated atrial septal defects in the oval fossa diagnosed in infancy

OBJECTIVE Our aim was to investigate the change in diameter of holes within the oval fossa, and the role of aneurismal formation in reducing the size of the hole, in patients diagnosed during infancy with isolated defects in the floor of the fossa. PATIENTS In a retrospective study, we included 100 patients diagnosed during the first year of life with an isolated defect in the floor of the oval fossa who had subsequently been observed for at least 5 years. There were 56 females and 44 males. They had been admitted to hospital because of a murmur in 65, heart failure in 9, and other reasons in 17. The remaining 9 patients were referred from other institutions with an established diagnosis of defects within the oval fossa. Patients were grouped according to the size of the deficiency in the floor of the fossa. Defects of diameter less than 5 mm were considered to be small, and 20 patients had such defects. Medium sized defects were judged to be between 5 and 8 mm, with 26 patients fulfilling this criterion, with the other 54 patients having large defects with diameters greater than 8 mm. RESULTS The overall spontaneous rate of closure was 27%. Of those with medium defects, half closed spontaneously, but only 7.5% of those with large defects showed such closure. Of the patients who were diagnosed with heart failure, 9 had defects measuring 7 mm, and of these, 6 required surgical closure, 1 patient had spontaneous closure, while the defect became smaller in the remaining 2. On the other hand, in 9 out of 10 patients who had aneurysms, the diameter of the defect was between 7 and 15 mm. Amongst these patients, the defect closed spontaneously in 3, and reduced in size in the others. CONCLUSION When holes within the oval fossa measure 8 mm or below, the majority of patients with experience either spontaneous closure or show decrease in size of the defect. In those with larger defects, the size usually increases, and surgery is needed for closure. If there is aneurismal formation, however, even when the defect measures more than 8 mm, the defect usually closes on its own or gets smaller.

Echocardiographic follow-up of congenital aortic valvular stenosis.

We investigated the morphology of the stenotic aortic valve, the progression of the stenosis, and the onset and progression of aortic regurgitation (AR) in patients with congenital aortic valvular stenosis (AVS). The medical records of 278 patients with AVS were reviewed, with the patients with concomitant lesions besides AR excluded. Very mild aortic stenosis was defined as a transvalvular Doppler peak systolic instantaneous gradient (PSIG) less than 25 mmHg, mild stenosis as 25–49 mmHg, moderate stenosis as 50–75 mmHg, and severe stenosis as more than 75 mmHg. The mean age of the patients was 4.9 ± 4.3 years (range, 3 days to 15 years), and 203 (73%) were male. The number of the cusps was determined with two-dimensional echocardiography in 266 patients (95%): unicuspid in 3 patients (1%), bicuspid in 127 patients (48%), and tricuspid in 136 patients (51%). A total of 192 of all patients were followed for 2 months to 14.6 years (mean 4.2 ± 3.3 years) with medical treatment alone. Among 72 patients with very mild stenosis at initial echocardiographic examination, 20% had mild, 3% moderate, and 1% severe stenosis after a mean period of 3.7 years. In 70 patients with mild stenosis at initial echocardiographic examination, 28% had moderate and 9% severe stenosis after a mean period of 5 years. Among 44 patients with moderate stenosis at initial echocardiographic examination, 36% had severe stenosis after a mean period of 3.7 years. Among 192 patients, 40% had AR (3% trivial, 28% mild, and 9% moderate) at initial echocardiographic examination. After a mean period of 4.2 years, 58% of the patients had AR (13 % trivial, 25% mild, 16% moderate, and 4% severe). There was not statistically significant difference between catheterization peak systolic gradients (47 ± 16 mmHg) and Doppler estimated mean gradients (45 ± 9 mmHg) (p = 0.53), whereas Doppler PSIGs (74.9 ± 15.7 mmHg) were higher than catheterization peak systolic gradients (p < 0.0001) in 25 patients who were studied in the catheterization lab. Patients with very mild stenosis may be followed with a noninvasive approach every 1 or 2 years, and an annual follow-up is suggested for patients with mild stenosis. Nearly one-third of patients with moderate stenosis at initial echocardiographic examination had severe stenosis after a mean period of 3.7 years. Therefore, we recommend, that patients with moderate stenosis undergo noninvasive evaluation every 6 months. Doppler estimated mean gradient is very useful in predicting the need for intervention in children with AVS.

Teratogenicity of Antiepileptic Drugs

Objective Antiepileptic drugs (AED) have chronic teratogenic effects, the most common of which are congenital heart disease, cleft lip/palate, urogenital and neural tube defects. The aim of our study is to examine teratogenic effects of AED and the correlation between these malformations and AED in single or multiple pregnancies. Methods This is a retrospective study of malformations in children born to mothers currently followed up by our outpatient clinics who used or discontinued AED during their pregnancy. Their children were then investigated using echocardiography, urinary ultrasound, cranial magnetic resonance image, and examined by geneticists and pediatric dentists. Results One hundred and seventeen children were included in the study. Ninety one of these children were exposed to AED during pregnancy. The most commonly used AED were valproic acid and carbamazepine in monotherapy. The percentage of major anomaly was 6.8% in all children. Dysmorphic features and dental anomalies were observed more in children exposed especially to valproic acid. There were 26 mothers with two and four mothers with three pregnancies from the same fathers. No correlation was found between the distribution of malformations in recurring pregnancies and AED usage. Conclusion Our study has the highest number of dysmorphism examined in literature, found in all the children exposed to valproic acid, which may account for the higher rate of facial dysmorphism and dental anomalies. On lower doses of valproic acid, major malformations are not seen, although the risk increases with polytherapy. Our data also indicate possible effects of genetic and environmental factors on malformations.