Abstract
We performed general anesthesia for a lip repair and palatoplasty in a patient with left ventricular hypoplasia following a Glenn procedure. Preoperative examination revealed hemorrhagic diathesis, hypoxemia, and secondary polycythemia. After completion of the palatoplasty, hypoxemia and intraoral bleeding were observed, and reintubation was required. The bleeding risk was likely increased in this patient due to several factors including the surgical procedure and concurrent antithrombotic therapy. In conclusion, the risks associated with hypoxemia and increased bleeding must be considered for the safe provision of general anesthesia during palatoplasty procedures in patients with cyanotic heart disease.
Keywords: Glenn procedure, Lip repair, General anesthesia, Palatoplasty, Cyanotic heart disease
In patients with single ventricle heart defects, the Glenn procedure is performed to help alleviate cyanosis by rerouting blood flow from the superior vena cava directly to the pulmonary arteries and is completed prior to additional repair with the Fontan procedure.1,2 Glenn circulation causes the blood from the inferior vena cava to mix with that in the pulmonary vein, leading to hypoxemia, cyanosis,3 and secondary polycythemia.4 We performed lip repair and palatoplasty under general anesthesia in a 1-year-old patient with left ventricular hypoplasia who had previously undergone a Glenn procedure.
The patient was diagnosed with bilateral cleft lip and palate, aortic coarctation, ventricular septal defects, left ventricular hypoplasia, single ventricular disease, and aortic stenosis. Pulmonary artery banding was performed 14 days after birth. Additionally, Norwood and Glenn surgeries were carried out at 4 and 11 months after birth, respectively.
The patient underwent lip repair at the age of 14 months (height 72.3 cm; weight 7.81 kg) and a palatoplasty at the age of 22 months (height 78.7 cm; weight 8.95 kg). Preoperative examination revealed hemorrhagic diathesis, hypoxemia, and secondary polycythemia. Hemoconcentration (14.6–15 g/dL) due to chronic hypoxemia was confirmed on preoperative examination. Thus, supplemental oxygen was administered via nasal cannula at 0.5 l/min, maintaining the SpO2 between 80 and 90%. The patient had been on aspirin for antiplatelet therapy, but heparin was substituted prior to the surgery.
General anesthesia during the lip repair was induced using sevoflurane (5%), midazolam (1 mg), fentanyl (10 μg), rocuronium (7 mg), and a continuous infusion of remifentanil (0.2 μg/kg/min). Anesthesia was maintained using fentanyl (56 μg) and sevoflurane (1.5–2%) in oxygen (FiO2 = 0.47). A total 5.8 mL of lidocaine with epinephrine was used for infiltration anesthesia. Low blood pressure was treated by administration of an α-1 adrenergic agonist or volume expansion with infusion to maintain the pulmonary blood flow. In addition, pulmonary blood flow was maintained by increased administration of oxygen and mild hyperventilation, optimizing pulmonary vascular resistance and oxygenation. The anesthetic management for the palatoplasty procedure mirrored that used for lip repair. However, after completion of the palatoplasty, hypoxemia and intraoral bleeding were observed, and emergent reintubation was required. The patient was managed in the ICU after surgery and extubated the next day. No additional bleeding-related complications were observed prior to the patient's discharge.
Bleeding is one of the most common postoperative complications found in patients who undergo palatoplasty procedures. The risk of bleeding further increases in patients on anticoagulant or antiplatelet therapy. Our patient had been treated with aspirin as the antithrombotic regimen; however, after hospital admission, it was substituted for heparin, which was started 1 week prior to surgery. Furthermore, intraoral bleeding can lead to airway loss and hypoxemia, which are especially problematic for patients with left ventricular hypoplasia. Therefore, it is important to carefully assess bleeding before extubation.
In conclusion, the risks of increased pulmonary vascular resistance, hypoxemia, and increased bleeding must be considered to provide safe general anesthesia during palatoplasty procedures in patients with cyanotic heart disease.
Acknowledgments
This research was originally published in the Journal of the Japanese Dental Society of Anesthesiology, 2019;47(2):65–67.
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