Anesthetic implications of elective cesarean section in a parturient with Noonan syndrome and complex cardiomyopathy: A case report

Noonan syndrome (NS) is an autosomal dominant multisystem disorder characterized by congenital cardiac defects, craniofacial anomalies, and hematological abnormalities. With an incidence of 1 in 1000 to 2500 live births, NS is one of the most common syndromes associated with congenital heart disease, second only to trisomy 21. Pregnant patients with NS present unique anesthetic challenges due to the syndrome’s diverse manifestations and the physiological changes of pregnancy. This report describes a 28-year-old woman with NS who underwent elective cesarean delivery at 36 weeks of gestation. Her medical history included repaired congenital heart defects, pulmonary stenosis, severe scoliosis, and asthma. Spinal anesthesia was successfully employed using a low-dose combination of local anesthetics and opioids to achieve effective sensory block while maintaining hemodynamic stability. The patient’s normal coagulation profile allowed neuraxial anesthesia to be a feasible option despite potential technical difficulties associated with spinal abnormalities. This case underscores the importance of a multidisciplinary approach to pregnant patients with NS, including comprehensive preoperative evaluation and individualized anesthetic planning. Neuraxial anesthesia can be a safe and effective option when tailored to the patient’s specific physiology, ensuring optimal maternal and fetal outcomes while minimizing risks.