Anesthetic management of a child with Holt-Oram syndrome with a pacemaker in situ undergoing urethroplasty

Dear Editor, Holt-Oram syndrome (HOS) is a rare autosomal dominant genetic disorder, resulting in atrial septal defect (ASD), ventricular septal defect (VSD), conduction abnormalities, and upper limb malformations (radial ray defects, polydactyly, or complete limb absence).[1] The anesthetic considerations include difficult vascular access, arrhythmias, and hemodynamic instability, requiring meticulous perioperative planning.[2] In this report, we discuss the anesthetic management in a HOS patient with left upper limb hypoplasia and radial ray defect [Figure 1] with a permanent pacemaker in situ, scheduled for urethroplasty.Figure 1: (a and b) Left upper limb hypoplasia and radial ray defectA 12-year-old (40 kg) male, with HOS, with penoscrotal hypospadias and urethral duplication was scheduled for urethroplasty. He had undergone surgical closure of large ASD with bidirectional left-to-right shunt and moderate muscular VSD at 6 years of age. After 2 weeks, he developed complete heart block, necessitating a permanent pacemaker in ventricular demand pacing, ventricular sensing, inhibited response, and rate modulation mode. Preoperatively, the patient’s functional capacity was good with no cardiac complaints. All routine blood investigations were normal. Electrocardiography revealed a paced ventricular rhythm, with widened QRS complexes and visible pacing spikes. Echocardiography revealed a normal study. With informed consent, the pacemaker mode was changed to ventricular pacing, no sensing, and no response to sensing mode to prevent electrocautery interference. Intraoperatively, defibrillation pads were placed in an anteroposterior direction to enable rapid defibrillation or cardioversion in case of life-threatening arrhythmias, triggered by surgical stress, anesthesia, or electrocautery. After premedication (midazolam 0.025 mg/kg), the child was induced with fentanyl (5 mcg/kg), propofol (1 mg/kg), and rocuronium (0.6 mg/kg). I-gel size 3 was inserted, ensuring controlled ventilation. An arterial line was inserted for invasive blood pressure monitoring. Intermittent phenylephrine boluses (0.25 mcg/kg) were used to keep the mean arterial pressure above 65 mmHg. Intravenous paracetamol (15 mg/kg) was given for analgesia. Intraoperative fluids were given judiciously. The procedure lasted 3 hours. The child was reversed with neostigmine (2 mg) and glycopyrrolate (0.4 mg) and extubated smoothly. Our case is unique as the patient had already undergone ASD and VSD repair, followed by complete heart block, requiring a permanent pacemaker in situ. The pacemaker’s pulse generator was implanted in the child’s abdomen behind the anterior rectus sheath, <15 cm from the surgical site, posing additional challenges, including potential electromagnetic interference and pacemaker monitoring. To maintain cardiovascular stability throughout the surgery, we preferred opioid induction and I-gel, which prevented the laryngoscopic response of intubation. As these patients have impaired autonomic compensation, a direct-acting vasopressor (phenylephrine) was preferred to maintain adequate perfusion. Ultrasound is the best modality to achieve venous cannulation due to hypoplastic peripheral vessels. To conclude, this case highlights the complex anesthetic management in patients with HOS. Preoperative assessment, optimizing pacemaker function, and vigilant intraoperative monitoring were essential in ensuring a successful outcome. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.