Case Report: Aarskog-scott syndrome caused by FGD1 gene variation: A family study.

BACKGROUND Percutaneous transvenous lead extraction (TLE) of cardiac implantable electronic devices can be performed with a high success rate. However, TLE has its limitations and challenges. Recognizing the challenges at an early stage during the procedure is vital for appropriate patient management. We present a challenging case of implantable cardioverter-defibrillator (ICD) lead extraction in which we aborted TLE in favor of elective surgical extraction (SE). This potentially prevented a major catastrophic complication of vascular tear, which would have required an emergent thoracotomy. CASE REPORT A 37-year-old woman with history of hypertrophic cardiomyopathy had a primary prevention dual-chamber ICD implant in 2001 and underwent right ventricular ICD lead revision in 2009 due to lead fracture. In 2019, she was again found to have right ventricular ICD lead malfunction. TLE was attempted, but no meaningful progression could be made despite using multiple extraction tools. Therefore, TLE was aborted in favor of SE. During elective SE, significant adhesions were noted, and the innominate vein was completely avulsed during removal of the leads, requiring venous reconstruction by the vascular surgery team. After SE and vascular reconstruction, an epicardial ICD system was placed, and the patient had an uneventful postoperative recovery. CONCLUSIONS This case report highlights the limitations of TLE and the importance of recognizing them in a timely manner. In all challenging cases, conversion to elective SE should be considered to avoid potential injuries warranting emergent surgical repair.