A rare ventriculoarterial connection: double outlet of both ventricles.

Ventriculoarterial connection is one of the important points of the segmental approach to congenital cardiac malformations. Double outlet of both ventricles is a rare form where both great arterial roots override the interventricular septum. In this article, we aimed to draw attention to this very rare form of ventriculoarterial connection by presenting an infant case diagnosed using echocardiography, CT angiography, and 3-dimensional modelling.

Cardiac resynchronization therapy in paediatric patients with congenital heart disease: single centre with 10 years of experience.

OBJECTIVES: In recent years, cardiac resynchronization therapy (CRT) has also started to be performed in the paediatric and CHD population. This study aimed to evaluate the efficacy of CRT in children with CHD. PATIENTS AND METHODS: Patients with CHD who underwent CRT treatment in our paediatric cardiology clinic between January, 2010 and January, 2020 were included in the study. Demographic findings, 12-lead electrocardiograms, echocardiograms, clinical characteristics, management strategies, and outcomes were reviewed systematically. RESULTS: The study population consisted of 18 CHD patients who had been treated with CRT for 10 years in our institution. The median age was 11 years (2.2-18 years) and the median weight was 39 kg (10-81 kg). Systemic ventricle was left ventricle in 13 patients, right ventricle in 4 patients, and 1 patient had single-ventricle physiology. CRT implantation indications were as follows: dysfunction after permanent pacemaker in 11 patients, dysfunction after left bundle branch block in 4 patients, and systemic ventricular dysfunction in 3 patients. CRT implantation techniques were epicardial (n = 13), hybrid (n = 4), and transvenous (n = 1) methods. QRS duration significantly decreased after CRT implantation (160 versus 124 m/second, p < 0.05). Median systemic ventricle ejection fraction (EF) significantly increased after the procedure (30 versus 50%, p < 0.05). Fourteen patients (78%) were responders, two patients (11%) were superresponders, and two patients (11%) were non-responders after the CRT treatment. One patient deceased during follow-up. Median follow-up duration was 40 months (6-117 months). CONCLUSION: When electromechanical dyssynchrony occurs in paediatric cases with CHD and developing heart failure, patients should be evaluated in terms of CRT to improve ventricular function. Alternative CRT therapy will be beneficial in these cases that do not improve clinically despite optimal medical treatment.

Comparison of trace element levels after cardiopulmonary bypass between cyanotic and acyanotic patients.

Trace elements are essential micronutrients for the human body. In this study, we evaluated the alterations in copper, chromium, manganese, selenium, magnesium, zinc, iron, arsenic, boron, and silicon levels in children with cyanotic and acyanotic CHD who underwent cardiac surgery with cardiopulmonary bypass. Participants were divided into the following three groups: patients acyanotic CHDs (n=34), patients with cyanotic CHDs (n=30), and healthy controls (n=30). Blood samples were collected before the surgery and 1 hour after the sternum was closed. Serum trace elements were determined by Inductively Coupled Plasma Optical Emission Spectrometer-ICAP 6000. The baseline serum arsenic, manganese, and zinc levels of both patient groups were lower compared with controls, but there was no significant difference between baseline serum trace element levels of cyanotic and acyanotic patients. In both the patient groups, there was a significant decrease in postoperative serum arsenic, boron, copper, and zinc levels, and a significant increase in postoperative serum iron and magnesium levels. Silicon levels increased in cyanotic patients. Alterations in trace element levels were in the same direction in cyanotic and acyanotic patients. Copper, zinc, and manganase replacement may be needed after on-pump cardiac surgery.

Alterations in antioxidant and oxidant status of children after on-pump surgery for cyanotic and acyanotic congenital heart diseases.

OBJECTIVE: Oxidative stress refers to an imbalance between reactive oxidative species and antioxidants. In this case-controlled, prospective, observational study, we investigated the total oxidant status, total antioxidant status, oxidative stress index, and albumin and C-reactive protein levels of children with cyanotic and acyanotic congenital heart diseases who had undergone on-pump cardiac surgery. METHOD: The study groups consisted of 60 patients with congenital heart disease, who were operated under cardiopulmonary bypass, and a control group of 30 healthy individuals. The patients were classified into two groups. Among them, one was a patient group that consisted of 30 patients with acyanotic congenital heart disease and the other group consisted of 30 patients with cyanotic congenital heart disease. In the patient groups, blood samples were collected before surgery and at one and 24 hours following surgery. In control groups, blood samples were collected once during hospital admission. RESULTS: No statistically significant differences were found between the groups in terms of baseline total oxidant status, total antioxidant status, and oxidative stress index values. Regarding the postoperative first-hour and 24-hour total oxidant status and total antioxidant status levels as well as oxidative stress index values, there were no significant differences between the groups, except for an increase in total antioxidant status levels (p=0.002) 24 hours after surgery in cyanotic patients. CONCLUSION: There was no difference between oxidative stress status of cyanotic and acyanotic congenital heart disease patients and healthy individuals. Oxidative stress status of cyanotic and acyanotic patients does not change after cardiac surgery under cardiopulmonary bypass.

Treatment of depression in an adolescent with cardiomyopathy and arrhythmia.

Patients with cardiomyopathy have a higher incidence of mood and anxiety disorders, resulting in greater probability for hospitalisation and increased risk for arrhythmia and death. We report a case of a 16-year-old boy with Danon disease, Wolff-Parkinson-White syndrome, and hypertrophic cardiomyopathy, who later developed depression and significant weight loss. The patient was successfully treated for his anxiety and depression with mirtazapine without any adverse cardiac effects.

Prolonged mechanical ventilation associated with hypothyroidism after paediatric cardiac surgery.

Hypothyroidism in patients undergoing congenital heart defect surgery is known to be possible. This generally temporary condition can progress as it involves yet other factors, increasing the patients' time to heal. The case presented here is that of a 5-month-old girl who was dependent in the long term on mechanical ventilation following cardiac surgery. After having been diagnosed with hypothyroidism, she was extubated on the fourth day of her hormone replacement therapy, and discharged from hospital on the tenth day.

Catheter ablation of idiopathic ventricular tachycardia in children using the EnSite NavX system with/without fluoroscopy.

OBJECTIVES: Curative therapy of idiopathic ventricular tachycardia remains a challenge in interventional electrophysiology. The aim of this study was to demonstrate the utility of an EnSite NavX system in the catheter ablation of idiopathic ventricular tachycardia in children. PATIENTS AND METHODS: In all, 17 children with idiopathic ventricular tachycardia underwent electrophysiological studies using the EnSite NavX system guidance. RESULTS: The mean patient age was 13 ± 2.4 years (range: 7.8-17.9) and the mean patient weight was 52.3 ± 11.9 kg (range: 32-75). The origin of ventricular tachycardia was in the right ventricular outflow tract in nine patients, in the left ventricle in six, near the bundle of His/right bundle branch in one, and in the left aortic cusp in one. The mean procedure and fluoroscopy times were 169.3 ± 43.2 minutes and 8 ± 10.8 minutes, respectively. No fluoroscopy was used in six patients. The mean radiation exposure was 33.1 ± 56.4 mGy. Acute success was achieved in 14 patients (82%). The focus of ventricular tachycardia was epicardial in two failed procedures. During a mean follow-up of 8.5 ± 7.6 months, ventricular tachycardia recurred in three patients, two of whom underwent a second procedure. Except for one patient who developed transient right bundle branch block, no complications were seen. CONCLUSION: Catheter ablation of idiopathic ventricular tachycardia in children can be performed safely and effectively with low fluoroscopy exposure using the EnSite NavX system.

The use of dornase alpha for post-operative pulmonary atelectasis after congenital heart surgery.

OBJECTIVE: To investigate the efficacy of dornase alpha, a mucolytic agent, in children who developed pulmonary atelectasis after congenital heart surgery. DESIGN: Retrospective case-control study. SETTING: Paediatric cardiac intensive care unit at a tertiary care hospital. PATIENTS: Between July, 2011 and July, 2012, 41 patients who underwent congenital cardiac operations and developed post-operative pulmonary atelectasis that was resistant to conventional treatment and chest physiotherapy. INTERVENTIONS: In all, 26 patients received dornase alpha treatment. As a control group, 15 patients were treated with conventional medications and chest physiotherapy. MAIN RESULTS: The median age of patients was 25.5 (3-480) days in the study group and 50.0 (3-480) days in the control group. A total of 15 (57.6%) patients in the study group and 8 (53.3%) patients in the control group were male. The median weight was 4.2 (2.9-14.2) kg and 4.0 (3.5-13.6) kg in the study and control group, respectively. In the study group, pulmonary atelectasis was diagnosed at a median period of 5 (2-18) days after operations, whereas in the control group atelectasis was diagnosed at a median period of post-operative 6 (3-19) days. In the study group, the median atelectasis score decreased from 3.4 (1-6) to 0.8 (0-3) (p = 0.001). The median pO2 level increased from 69 (17-142) mmHg to 89 (30-168) mmHg (p = 0.04). In addition, heart rate and respiratory rate per minute were significantly decreased (p < 0.05). There were no significant changes in these parameters in the control group. CONCLUSIONS: The use of dornase alpha can be effective for the management of pulmonary atelectasis that develops following congenital heart surgery.