Noonan syndrome is an autosomal dominant genetic disorder characterized by multisystem involvement and frequent cardiac anomalies such as hypertrophic cardiomyopathy (HCM), left ventricular outflow tract (LVOT) obstruction, pulmonary stenosis, and valve abnormalities. These clinical features significantly increase perioperative risk. We present the anesthetic management of a 3-month-old infant with Noonan syndrome undergoing epicardial implantable cardioverter-defibrillator (ICD) placement. The patient had a history of cardiac arrest, HCM, LVOT obstruction, patent foramen ovale, and mild valvular regurgitations. General anesthesia was induced with midazolam, fentanyl, and rocuronium; maintenance was achieved with remifentanil infusion and sevoflurane. Invasive arterial monitoring and bispectral index (BIS) were used. Intraoperative hemodynamics remained stable, and the patient was transferred to the intensive care unit (ICU) intubated and sedated. However, postoperative deterioration occurred due to necrotizing enterocolitis on day 2, and despite resuscitative efforts following cardiac arrest on postoperative day 5, the patient died. This case highlights the complexity of anesthetic care in Noonan syndrome due to difficult airway features, severe cardiac involvement, and risk of perioperative complications.
Selçuk et al. (Tue,) studied this question.