A new association of multiple congenital anomalies/mental retardation syndrome with bradycardia-tachycardia syndrome: a case report

Congenital bradycardia-tachycardia syndrome is a rare disorder. Its association with multiple congenital anomalies/mental retardation (MCA/MR) syndrome is exceptional.

We report a case of a new association of MCA/MR with bradycardia-tachycardia syndrome in an 18-year-old Indian man. This syndrome is characterized by mental retardation with delayed development of milestones, progressive scoliosis, cryptorchidism, asymmetrical limbs involving both the upper and lower limbs, sleep apnea syndrome, bradycardia-tachycardia syndrome and Dandy-Walker syndrome. Our patient was admitted for septoplasty with adenoidectomy. Patients with MCA/MR with bradycardia-tachycardia syndrome pose a unique challenge to the anesthesiologist. Establishing a good rapport with these patients is imperative. In addition to that, the anesthesiologist should anticipate the difficulty in intubation and rhythm abnormalities during the peri-operative period. Bradycardia or sinus arrest is a well-known complication during the induction and maintenance of anesthesia. Lignocaine should be used with caution in patients with bradycardia-tachycardia syndrome. Monitoring of ventilation parameters (end-tidal CO₂, SPO₂, airway pressure) is essential as these patients are prone to develop pulmonary artery hypertension secondary to sleep apnea syndrome.

Based on our clinical experience in detailed pre-operative evaluation and planning, we would emphasize peri-operative anticipation and monitoring for dysrhythmias in patients with MCA/MR and bradycardia-tachycardia syndrome undergoing any surgical procedure.