Bilateral thoracoscopic sympathectomy for cardiac denervation in pediatric population: Does Kuntz nerve cauterization have an impact on success?

INTRODUCTION: Cardiac sympathetic denervation (CSD) is an effective procedure for the treatment of inherited channelopathies. Left CSD has traditionally been recommended as neuromodulation to prevent arrhythmia episodes; however, it is thought that bilateral sympathectomy in combination with Kuntz ablation may have additional effects effective due to the anatomical variability of preganglionic sympathetic fibers. The aim of the study was to share our single-center clinical experience with bilateral thoracoscopic sympathectomy for cardiac denervation in different groups of pediatric patients with malignant arrhythmias. METHODS: Fourteen patients (seven with CPVT, five with LQTS, one with resistant ventricular tachycardia as a sequela of myocarditis, and one with cardiomyopathy and atrial tachycardia) underwent bilateral thoracoscopic sympathectomy for cardiac denervation. RESULTS: In all patients, arrhythmia episodes persisted despite medical therapy, and patients with implantable cardioverter-defibrillator received appropriate therapies. The rate of appropriate therapies was 3.25 per year (1-5 per year) in the year before sympathectomy. No major complications related to the procedure were observed in any of the patients. The QTc interval of LQTS patients decreased from 506.2 ± 16.9 ms before the procedure to 476 ± 28.8 ms after the procedure. The mean duration of follow-up after the procedure was 23.3 months (11-47 months). Only two patients received single episodes of therapy 12 and 22 months after CSD, and one patient had arrhythmic events due to noncompliance to medical therapy. CONCLUSION: Due to the anatomical variability of preganglionic fibers bilateral CSD with Kuntz nerve ablation effective treatment and is a safe option.

Transcatheter closure of patent vertical vein after repair of total anomalous pulmonary venous connection: a case series.

BACKGROUND AND OBJECTIVES: Total anomalous pulmonary venous connection is a rare cyanotic CHD that requires surgical repair. An unligated vertical vein after total anomalous pulmonary venous connection surgery may help to decrease the episodes of post-operative pulmonary hypertensive crises, low cardiac output syndrome, and mortality. The aim was to assess long-term outcome and our post-operative transcatheter vertical vein closure experiences in five patients with repaired total anomalous pulmonary venous connection patients. METHODS: A retrospective study was conducted in five cases with an unligated vertical vein following repair of supra-cardiac total anomalous pulmonary venous connection at our hospital from 2011 through 2018. Patients characteristics, cardiac catheterisation findings, surgical, and transcatheter procedural details were retrospectively analysed. RESULTS: Transcatheter closure of the unligated vertical vein was technically successful in all the patients. Procedure-related complications were not observed in any of the patients. No long-term complication was found. CONCLUSIONS: We suggest that transcatheter closure of the patent vertical vein is an effective and well-tolerated alternative to the surgical approach.

A rare coincidence: the long QT syndrome and cardio-facio-cutaneous syndrome.

Cardio-facio-cutaneous syndrome is a genetic anomaly characterised by craniofacial dysmorphia, developmental retardation, skin lesions, mental retardation/learning disability, and cardiac malformations. Cardio-facio-cutaneous syndrome rarely causes arrhythmias and has not been previously associated with long QT in the literature. With this report, it was aimed to draw attention to a different presentation of the long QT syndrome.

Acute coronary syndrome in an adolescent with aortic root replacement (Bentall).

Acute coronary syndrome is a cause of cardiac-induced chest pain and rarely seen among childhood. It should be kept in mind in patients presenting with typical chest pain with a history of CHD and/or surgery, as this will lead to life-threatening complications and death. We present an adolescent with a history of Bentall operation who had acute coronary syndrome owing to coronary thrombosis as a result of inappropriate drug use.

Femoral venous haemostasis in children and young adults using the 'figure-of-eight' suture technique.

AIM: The aim of our study was to evaluate the safety and efficiency of the 'figure-of-eight' suture among children and young adults with congenital heart defects who underwent interventional procedures, in patients with structurally normal hearts who underwent electrophysiological study and in haemodynamically impaired children and newborns. We also reported a novel femoral haemostasis method in patients with a central catheter by modifying the 'figure-of-eight' suture around the catheter for haemorrhage control. METHOD: Between 2015 and 2018, a total of 100 'figure-of-eight' sutures were performed in 90 patients (48 males, 42 females) where the median age was 12.5 years (minimum 3 days, maximum 22 years). The procedures were diagnostic angiography (n = 6), radiofrequency and/or cryoablation (n = 7) and interventional procedures (n = 87). RESULT: Haemostasis was achieved in 89 of 90 patients. Haemostasis could not be achieved in one malnourished patient due to lack of subcutaneous tissue. There were no major complications. A bullous skin lesion and minor bleeding were the only complications seen in two patients. A central catheter was inserted in eight patients using the modified 'figure-of-eight' suture technique. CONCLUSION: The 'figure-of-eight' suture is a safe and effective method for femoral venous haemostasis in patients who require large sheaths for procedures, in those using high-dose heparin and in haemodynamically unstable children who need cardiac catheterisation.