Background
Catecholaminergic polymorphic ventricular tachycardia is a type of malignant arrhythmia that is triggered by adrenergic stimulation without detectable structural defects. The prevalence of disease is estimated to be 1:10,000 and is associated with sudden death. Common treatment options include Beta Blocker, ICD, and recently left and bilateral sympathectomy. In the case for bilateral sympathectomy in pediatric population, lung isolation strategies are challenging due to the patients' small size and equipment availabilities.
Case Description
7-year old 23 kg otherwise healthy male with a history of cardiac arrest secondary to catecholaminergic polymorphic ventricular tachycardia presents for bilateral video assisted thoracoscopic sympathectomy. Labs were within normal limits. After considering the risks and benefits of implantable cardioverter defibrillator versus bilateral sympathectomy, it was decided that sympathectomy was the best option. Given the patient’s small size, lung isolation strategies were challenging due to size limitations with available equipment. After careful consideration, we decided to proceed with the case with a pediatric endobronchial blocker.
The patient was brought into the operating room after premedication with intravenous midazolam. Standard ASA monitors were applied. In addition, external defibrillator pads were placed prior to induction. Smooth intravenous induction was accomplished with propofol, fentanyl and rocuronium. We attempted to place a 6.0 mm ID cuffed ETT, however, we were unable to pass the ETT easily. A 5.5 mm ID cuffed ETT was placed atraumatically with the cuff deflated and minimal leak was detected at 20 mmHg. A 22G radial arterial line and large peripheral intravenous access were placed after successful intubation.
We then proceeded with placement of a 5 Fr Arndt endobronchial blocker into the right main bronchus via direct visualization using a 2.8 mm diameter pediatric bronchoscope. The blocker position was reconfirmed after positioning the patient left lateral. One lung ventilation was initiated with the inflation of the endobronchial blocker balloon under direct visualization. The patient tolerated one lung ventilation on pressure control and FiO2 at 50% without issues. Tidal volume under single lung ventilation was around 120-150 ml with peak airway pressure of 14-15 mmHg. The surgeon reported excellent surgical field exposure and right sympathectomy was completed successfully without complications.
The patient was then repositioned right lateral and re-prepped and re-drapped for left sympathectomy. The endobronchial blocker was repositioned into the left main bronchus under direct visualization with the pediatric bronchoscope. One lung ventilation was initiated with the inflation of the endobronchial blocker balloon under direct visualization. Left sympathectomy was completed successfully without complications. The patient tolerated the procedures well and was extubated at the end of the procedure without event.
The patient was followed closely by cardiology postoperatively. He is doing well without new episodes of syncope or cardiac arrest.
Discussion
Lung isolation techniques in pediatric population can be challenging. Many different techniques are available. This includes utilization of single lumen tube, double lumen tube, univent tube, bronchial blocker, fogarty catheters to name a few. A good understanding of available equipment and careful planning will improve success rate when dealing with these challenging situations.
