Multidetector Computed Tomographic Angiography Imaging of Congenital Pulmonary Venous Anomalies: A Pictorial Review.

Congenital pulmonary venous anomalies are not uncommon that can occur either in isolation or in association with different forms of congenital heart disease. Clinical presentation of these anomalies may vary from the relatively benign single anomalous partial pulmonary venous return to life-threatening critical obstructed total anomalous pulmonary venous return. Accurate delineation of these anomalies and accompanied cardiovascular anomalies are crucial to guide decision making in these patients. Low-dose high-pitch dual-source 256-detector multidetector computed tomographic angiography is a fast and reliable imaging modality allowing comprehensive noninvasive anatomic imaging in neonates and children with congenital pulmonary venous anomalies with lower radiation doses and should be preferred for these patients after transthoracic echocardiography.

Cryoablation with an 8-mm-Tip Catheter for Right-Sided Accessory Pathways in Children.

BACKGROUND: Cryoablation is increasingly utilized in children because of its safety profile. Recently, larger catheter tips have been more widely used to improve long-term success rates. The aim of this study was to assess the safety and efficacy of 8-mm-tip catheters for cryoablation of right-sided accessory pathways (APs) in children. METHODS: Electrophysiological procedures were performed using the EnSite™ system (St. Jude Medical Inc., St. Paul, MN, USA). RESULTS: Between July 2010 and July 2014, 54 patients (mean age: 13.1 ± 3.7 years) underwent cryoablation using an 8-mm-tip catheter. In 18 of 54 (33%) patients where an 8-mm-tip catheter was the first-choice catheter, the success rate was 18 of 18 (100%). There was a history of previous failed attempts or recurrence with radiofrequency ablation and/or 6-mm-tip cryoablation in 36 of 54 (67%) patients. The success rate in these patients was 24 of 36 (67%). No fluoroscopy was used in 34 of 54 procedures. The recurrence rate was six of 42 (14%) during a mean follow-up period of 32 ± 15 months. In one patient, transient atrioventricular block occurred. CONCLUSIONS: Cryoablation with an 8-mm-tip catheter for right-sided APs in children who weigh over 40 kg appears to be safe and acutely effective in cases where conventional ablation methods fail and also as a first choice for ablation procedure. However, the recurrence rate still seems to be high.

Factors affecting perioperative mortality in tetralogy of Fallot.

BACKGROUND: We evaluated the preoperative, operative and postoperative risk factors affecting early mortality in patients who underwent total correction of tetralogy of Fallot (TOF). METHODS: One hundred and twenty-two TOF patients who underwent reparative surgery between January 2010 and November 2013 were enrolled in the study. RESULTS: Mean patient age and weight was 2.3 ± 2.5 years and 11.3 ± 6.4 kg, respectively. Cardiac catheterization was performed in 101 patients (83%),and coronary anomalies were found in 11 patients. Mean McGoon index, pulmonary annulus z-score, main pulmonary artery z-score, left pulmonary artery z-score and right pulmonary artery z-score were 2.0 ± 0.4, -1.85 ± 1.54, -2.84 ± 2.06, 1.17 ± 1.73, and 0.74 ± 1.57, respectively. Total reparative surgery with a transannular patch was performed in 97 patients (79.6%); the rest underwent valve-sparing surgery. Median duration of postoperative mechanical ventilation, intensive care and hospital stay were 19 h, 3 days and 9 days, respectively. Extracorporeal membrane oxygenation (ECMO) was required in 10 patients in the postoperative early period. Arrhythmias occurring in the early postoperative period were junctional ectopic tachycardia (n = 13), complete atrioventricular block(n = 10; permanent epicardial pacemaker implanted in four) and ventricular tachycardia (n = 4). Nine patients died in the early postoperative period (7.3%). Parameters found to be associated with increased mortality were low preoperative oxygen saturation; high right ventricular/aortic pressure ratio immediately after surgery; presence of coronary anomaly; requirement of postoperative ECMO; and pacemaker (P = 0.02, P = 0.04, P = 0.01, P = 0.0001, P = 0.03, respectively). CONCLUSIONS: Poor preoperative oxygenation, presence of coronary anomaly, complete AV block in the early postoperative period, high RV pressure and requirement of ECMO appear to be the most significant factors that affect early mortality in the surgical treatment of TOF. Appropriate preoperative assessment, correct surgical strategies and attentive intensive care monitoring are required in order to reduce mortality.

Successful transvenous lead extraction using the Evolution System in a 17-kg child.

Despite technological advancements in the field of pacemaker lead extraction, available data on pediatric patients is limited, and risk of failure and severe complications remains high. In this report, we present the case of a male patient who, at five months old, had been transvenously implanted with a single-chamber ventricular pacemaker due to complete atrioventricular block. At 7-year of age, the patient was referred to us with growth-related lead tension, severe tricuspid insufficiency, and weak battery. We extracted the lead using the Evolution System and replaced the unit with a dual-chamber pacemaker with a SelectSecure lead. This new system can be used for lead extraction even in low-weight pediatric patients with congenital heart disease. Using a thin, lumenless SelectSecure lead appears to reduce the risk of venous obstruction.

The use of flecainide in critical neonates and infants with incessant supraventricular tachycardias.

OBJECTIVE: This study aimed to evaluate the efficacy of flecainide therapy in neonates and infants with drug resistant incessant supraventricular tachycardia. METHODS: The study included 11 neonates and infants who received medical and/or ablation therapy between January 2010 and December 2013. Mean patient age and weight were 101.6 ± 96 days and 5.3 ± 1.9 kg respectively. Of the 12 patients, 5 underwent ablation between January 2010 and December 2011, and 6 were treated medically between January 2012 and December 2013. Mean follow-up time was 18 months (6 months-4 years). RESULTS: The antiarrhythmic agent flecainide only became available in Turkey in 2012, and the most noteworthy point was its addition to the therapy administered prior to ablation (adenosine, esmolol-propranolol, propafenone, amiodarone and cardioversion). In all 6 patients admitted between January 2012 and December 2013, refractory SVT was successfully treated with the administration of a triple therapy regimen of esmolol-propranolol, amiodarone and flecainide. One patient with myocarditis developed an atrial flutter complicated by a concealed accessory pathway and was put on extracorporeal membrane oxygenation (ECMO) support due to cardiopulmonary failure. The SVT was terminated, but the patient died on the fifteenth day of ECMO support. One patient with recurrent tachycardia, who had previously undergone ablation for a complex cardiac anomaly and Wolf-Parkinson-White syndrome, was treated with ablation again. No recurrence of tachycardia was observed in any of the other 9 patients. CONCLUSION: It appears that the use of propranolol-esmolol and amiodarone combined with flecainide in the medical treatment of drug-resistant SVT may reduce the need for ablation in critical neonates and infants.

Holter electrocardiography findings and P-wave dispersion in pediatric patients with transcatheter closure of atrial septal defects.

BACKGROUND: This study aimed to determine the frequency of postintervention arrhythmia and factors associated with the development of arrhythmia, including the correlation between arrhythmia and P-wave dispersion, and the effects of the latter on transcatheter closure of atrial septal defects (ASDs). METHODS: Holter ECG recordings were performed before and after the intervention and 6 and 12 months later in 47 of the 59 patients who had undergone transcatheter ASD closure and once in the healthy control subjects. RESULTS: A statistically significant correlation was identified between the patients' arrhythmia grade according to Lown's system and each of the following: the number of defects, the size of the atrioventricular valve rim, the presence of an atrial septal aneurysm. CONCLUSION: The frequency of arrhythmia increases after transcatheter ASD closure, gradually decreases within the next year, and is most frequently of a benign nature. Lown's arrhythmia grading of patients occluded with either the Amplatzer septal occluder (ASO) or the Cardio-O-Fix septal occluder (CSO) were compared, and the arrhythmia frequency was higher with the latter. One day after the intervention, the P maximum (Pmax ) and the P dispersion(Pdis ) values were not increased but in fact slightly reduced in patients occluded with either ASO or CSO. An improvement in the electrical system resulting from early anatomical and mechanical healing following transcatheter ASD occlusion may explain the reduction in the Pmax and Pdis values.

Occlusion of the left superior vena cava-coronary sinus connection in a child with Glenn dysfunction by the transcatheter approach.

A 14-year-old male patient presented with cyanosis and tiredness. The patient had undergone a Glenn procedure at age 12 following the echocardiographic determination of a double inlet left ventricle, ventriculoarterial discordance, moderate valvular-subvalvular pulmonary artery stenosis, non-restrictive inlet ventricular septal defect and right ventricle hypoplasia; his oxygen saturation was 70%. Echocardiography evaluation showed retrograde flow from the vena cava superior to the innominate vein and a left superior vena cava (LSVC) opening into the coronary sinus (CS). Here, we report the case of a patient evaluated for Glenn dysfunction in whom an increase in oxygen saturation was observed following transcatheter occlusion of the LSVC-CS connection using an Amplatzer septal occluder.

Cryoablation of anteroseptal accessory pathways in children with limited fluoroscopy exposure.

Due to its safety profile, cryoablation is used increasingly in pediatric patients, especially for septal arrhythmia substrates. Recent advances in electroanatomical-mapping technologies have resulted in a decrease or complete elimination of fluoroscopy exposure during catheter ablation procedures. The aim of this study was to assess the efficacy and safety of cryoablation of anteroseptal accessory pathways (APs) using electroanatomical-mapping system guidance with limited fluoroscopy exposure. A total of 24 patients underwent cryoablation of anteroseptal APs between July 2010 and April 2012. Cryomapping was performed with a 6 mm-tip catheter at -30 °C before the lesions were delivered. An 8 mm-tip catheter was used in one patient. The EnSite system (St. Jude Medical, St Paul, MN) was used in all procedures. The mean age was 11.9 ± 4.3 years. Acute success rate was 95.8 % (23 of 24). The mean procedure and cryoablation durations were 168 ± 58 min and 1,463 ± 525 s, respectively. Limited fluoroscopy was used only in 7 patients, and the mean fluoroscopy time was 1.7 ± 1.8 min (range 0.1-4 min) in these patients. Recurrence was noted in 2 patients (8.7 %) who underwent a second successful cryoablation procedure. The patient who underwent a failed attempt during the first procedure was successfully treated with a repeat procedure. The resultant long-term success rate was 100 % at a mean follow-up period of 14.2 ± 7.7 months. There were no complications except for transient atrioventricular block in one patient. Cryoablation of anteroseptal APs can be performed effectively and safely in children using a limited fluoroscopic approach with the help of electroanatomical-mapping systems.

Low-Dose Computed Tomographic Imaging of Partial Anomalous Pulmonary Venous Connection in Children.

BACKGROUND: In this study, we aimed to determine lobar distribution, drainage sites, and associated cardiovascular anomalies of partial anomalous pulmonary venous connection in pediatric patients using low-dose multidetector computed tomographic angiography. METHODS: Sixty-one cases (27 female, mean age: 4.7 years) with partial anomalous pulmonary venous connection diagnosed by multidetector computed tomographic angiography were included in this study. In all patients, multidetector computed tomographic angiography examinations were performed using dual-source 256-slice scanner without sedation. RESULTS: In 61 patients, 73 anomalous pulmonary veins were detected, 56 (77%) of them were right-sided and 17 (23%) were left-sided. Of 56 right-sided anomalous pulmonary veins in 49 patients, 38 (68%) drained into superior vena cava, eight (14%) into atriocaval junction, six (11%) into inferior vena cava, three (5%) into right atrium, and one (2%) into levoatriocardinal vein. Of 17 left-sided anomalous pulmonary veins in 12 patients, 16 (94%) drained into left innominate vein, and one (6%) into coronary sinus. Only seven (12%) patients had isolated partial anomalous pulmonary venous connection, whereas 54 (88%) patients had additional cardiovascular anomalies. The most common (66%) associated anomaly is atrial septal defect. The overall mean effective radiation dose was 1.12 mSv (range: 0.15-7.41 mSv), and it was 0.58 mSv (range: 0.15-0.73) in the patients younger than one-year old. CONCLUSIONS: The presence and course of the anomalous pulmonary veins and associated cardiovascular anomalies can be reliably detected by dual-source 256-slice multidetector computed tomographic angiography with low radiation doses.

Total Anomalous Pulmonary Venous Connection in Children: Preoperative Evaluation with Low-Dose Multidetector Computed Tomographic Angiography.

We report the results of our retrospective evaluation, from February 2011 through August 2014, of the anatomic features of total anomalous pulmonary venous connection (TAPVC) and its associated cardiovascular anomalies in a pediatric population. In all 43 patients under study (23 female; average age, 9 mo [range, 4 d-7.1 yr]), these examinations had been performed with a dual-source 256-detector scanner. The type of TAPVC, the presence of obstruction, and the association with other cardiovascular anomalies were investigated and recorded. In accordance with the absence or presence of these accompanying anomalies, patients were subdivided into 2 groups: isolated and complex. In the 43 patients, 22 (51%) TAPVCs were supracardiac, 10 (23%) were cardiac, 6 (14%) were infracardiac, and 5 (12%) were mixed. Obstruction was detected in 7 patients. Seventeen patients were in the isolated group and 26 in the complex group. The diagnostic agreements between multidetector computed tomographic angiographic and surgical results were 100% in both the isolated and complex groups. The overall average effective radiation dose was 0.66 mSv (range, 0.15-1.11 mSv); and it was 0.52 mSv (range, 0.12-0.72 mSv) in patients younger than 1 year of age. We conclude that computed tomographic angiography with a dual-source 256-slice multidetector scanner is a reliable imaging method that enables, despite lower radiation doses, the detailed and comprehensive anatomic imaging of TAPVC in neonates and children.

Heart rate variability improvement in children using transcatheter atrial septal defect closure.

OBJECTIVE: We evaluated autonomic behavior by examining heart rate variability (HRV) in the time domain and frequency domain in pediatric patients who underwent transcatheter closure of atrial septal defect (ASD). METHODS: A prospective study design was used. Holter ECG was performed in a control group of 30 healthy subjects and a group of 47 patients who underwent transcatheter ASD closure. ECG was taken one day before, one day after, and six months after the procedure to evaluate changes in the time domain [SDNN, rMSSD, NN, pNN50(%), and SDANN] and frequency domain (VLF, LF, HF, VHF, and LF/HF) in the patient group. Student's t-test was used to evaluate changes prior to and after the procedure. RESULTS: There were 28 females (60%) in the patient group and 21 females (70%) in the control group. The mean age and weight of the participants in the patient group were 9.61±4.72 years and 32.40±19.60 kg, respectively; the mean age and weight of the control subjects were 10.43±5.31 years and 32.83±13.00 kg, respectively. In both the time domain and frequency domain analyses, the patient group values were found to be lower than those in the control group prior to the procedure; the values in the patient group were found to approach the values in the control group following the procedure. By the sixth month, the values in the patient group reached the control levels with no statistically significant difference (SDNN: 145±0.84, 137.50±42.50; rMSSD: 72.18±48.22, 58.14±28.49; SDANN: 125.13±13.50, 122.40±41.06; VLF: 112.85±29.07, 114.41±98.39; LF: 50.40±24.09, 45.69±15.13; HF: 39.28±19.86, 44.29±13.14; VHF: 10.29±4.24, 9.99±6.47; LF/HF: 1.90±1.44, 1.24±0.81; p>0.05). CONCLUSION: The transcatheter closure of secundum ASDs was found to have a positive effect on HRV. Consequently, it may contribute to reduced mortality and morbidity. We can conclude that in children, HRV recovers approximately six months after transcatheter ASD closure.

Ductal stent implantation in tetralogy of fallot with aortic arch abnormality.

Stenting of patent ductus arteriosus is an alternative to palliative cardiac surgery in newborns with duct-dependent or decreased pulmonary circulation; however, the use of this technique in patients with an aortic arch abnormality presents a challenge. Tetralogy of Fallot is a congenital heart defect that is frequently associated with anomalies of the aortic arch and its branches. The association is even more common in patients with chromosome 22q11 deletion. We present the case of an 18-day-old male infant who had cyanosis and a heart murmur. After an initial echocardiographic evaluation, the patient was diagnosed with tetralogy of Fallot and right-sided aortic arch. The pulmonary annulus and the main pulmonary artery and its branches were slightly hypoplastic; the ductus arteriosus was small. Conventional and computed tomographic angiograms revealed a double aortic arch and an aberrant left subclavian artery. The right aortic arch branched into the subclavian arteries and continued into the descending aorta, whereas the left aortic arch branched into the common carotid arteries and ended with the patent ductus arteriosus. After evaluation of the ductal anatomy, we implanted a 3.5 × 15-mm coronary stent in the duct. Follow-up injections showed augmented pulmonary flow and an increase in oxygen saturation from 65% to 94%. The patient was also found to have chromosome 22q11 deletion.

Atrial fibrillation in a healthy adolescent after heavy smoking of contraband cigarettes.

The use of contraband cigarettes is a serious public health problem. We present a case of atrial fibrillation in a healthy adolescent suspected to be caused by smoking contraband cigarettes. A 15-year-old man was admitted to our emergency department experiencing syncope and palpitations. He was a cigarette smoker, but he had never smoked any illicit tobacco products before. He had finished a pack of counterfeit cigarettes (20 pieces) in 1.5 h. His electrocardiogram showed atrial fibrillation with a rapid ventricular response and irregular RR intervals. The patient had no history of alcohol use, surgery, palpitations, hypertension, chronic bronchitis, or any infectious diseases. His atrial fibrillation was converted to a normal sinus rhythm after the cardioversion treatment. Our patient was discharged from the pediatric cardiology service and advised to quit smoking cigarettes, strictly warning against illicit tobacco products. In conclusion, intensive smoking of counterfeit cigarettes may lead to occurrences of atrial fibrillation.

Utility of Exercise Testing and Adenosine Response for Risk Assessment in Children with Wolff-Parkinson-White Syndrome.

BACKGROUND: We aimed to determine the correlation between noninvasive testing (exercise stress testing [EST] and adenosine responsiveness of accessory pathway [AP] ) and invasive electrophysiology study (EPS) for assessment antegrade conduction of the AP in Wolff-Parkinson-White syndrome. PATIENTS AND METHOD: This prospective, observational study enrolled 40 children (58% male children, median age of 13 years, and median weight of 47.5 kg) with Wolff-Parkinson-White syndrome. Conduction through the AP to a cycle length of ≤250 ms was considered rapid or high-risk; otherwise, patients were nonrapid or low-risk. RESULTS: The sudden disappearance of the delta-wave was seen in 10 cases (25%) during EST. Accessory pathway was found to be high-risk in 13 cases (13/40, 32.5%) while the accessory path was identified as low-risk in 27 cases; however, six patients (15%) had blocked AP conduction with adenosine during EPS. Low-risk classification by EST alone to identify patients with nonrapid conduction in baseline EPS had a specificity of 93% and a positive predictive value of 90% (accuracy 54%). Blocked AP conduction with adenosine as a marker of nonrapid baseline AP conduction had a specificity of 93% and a positive predictive value of 84%. Finally, AP was adenosine nonresponsive in the majority of patients (28/30, 93%) with persistent delta-waves, 40% of those who had a sudden disappearance of delta-waves had an adenosine-responsive AP (P value: .028). CONCLUSION: Abrupt loss of preexcitation during EST and blocked AP conduction with adenosine had high specificity and positive predictive value for nonrapid and low-risk antegrade conduction during baseline invasive EPS. Successful risk stratification of pediatric patients with Wolff-Parkinson-White is possible through the use of EST and the adenosine responsiveness of AP.

The significance of transesophageal echocardiography in assessing congenital heart disease: our experience.

BACKGROUND: The rapid expansion of pediatric cardiovascular surgery, along with the increase in the number of centers and operations, has also increased the use of intraoperative transesophageal echocardiography (TEE). Preoperative TEE allows for the confirmation of diagnoses established by transthoracic echocardiography and angiography, and also identifies possible additional pathologic conditions. TEE is also used to follow up on surgical repairs, determine the need for reintervention, and evaluate myocardial performance and the need for inotropic treatment. Our intention is to share the TEE findings before and after intervention for congenital heart disease in patients who received operations at our center, and the conclusions reached. PATIENTS AND METHOD: From October 2009 to February 2013, 1008 patients underwent surgery for congenital heart disease. Of these, 265 were subjected to TEE in the operating room. Each patient entering the operating room with TEE planned had the echocardiography probe placed while intubated under general anesthesia. TEE studies used a GE Vivid S5. Different investigations, according to the patients' particular pathologic conditions, were also performed in addition to standard TEE studies. RESULTS: Of the 265 patients for whom surgery was indicated by transthoracic echocardiography and other appropriate examinations, 260 had the diagnosis confirmed by preoperative TEE and the indicated intervention was performed. For the remaining 5 patients (1.8%), the intervention plan was changed following preoperative TEE. Cardiopulmonary bypass was reinitiated in 12 (4.5%) patients because of residual defects identified by postoperative TEE. Thus, the preoperative plan was changed in 17 (6.4%) patients out of a total of 265 owing to preoperative and postoperative TEE findings. CONCLUSION: The use of intraoperative TEE in surgical centers for congenital heart disease allows for a significant reduction in mortality and morbidity. Intraoperative TEE performed by experienced pediatric cardiologists is therefore an absolute necessity.