Anesthetic Implications in Holt-Oram Syndrome Patients Undergoing Cardiac Procedures. Two case reports.

BackgroundHolt-Oram syndrome (HOS) is a rare genetic disorder, first described in 1960, It is characterized by upper limb deformities, congenital heart defects, and conduction abnormalities, posing significant anesthetic management challenges. This report presents two cases of HOS undergoing atrial septal defect (ASD) closure—one through a transcatheter approach and the other via surgical repair.Case PresentationCase 1: A 6-year-old female, weighing 18kg, diagnosed with HOS. The patient underwent transcatheter ASD closure under deep sedation with dexmedetomidine and ketamine. Anesthesia was maintained without complications, and the procedure was completed successfully, with the patient recovering well in the cardiac care unit.Case 2: A 3.5-year-old female, weighing 17.5kg, with HOS, presented with bilateral upper limb anomalies (absent distal radius, short humerus, absent thumb) and ASD. The patient underwent surgical ASD closure under general anesthesia. The procedure was uneventful, and the patient was successfully weaned from cardiopulmonary bypass, with no residual ASD or arrhythmias observed postoperatively.DiscussionAnesthetic management of HOS requires thorough preoperative assessment, including evaluation of conduction anomalies, chest wall deformities, and airway considerations. Malignant hyperthermia, though reported in isolated cases, was not observed in our patients, despite exposure to potential triggers. Arrhythmias, a common concern in HOS, were anticipated but not exacerbated during the procedures. Special attention was given to femoral arterial cannulation in the surgical case due to upper limb anomalies. The use of TEE was minimized in the transcatheter case to reduce the need for general anesthesia.ConclusionsAnesthetic management in HOS patients undergoing cardiac procedures necessitates a multidisciplinary approach with vigilant monitoring for potential complications. While the risk of arrhythmias and malignant hyperthermia requires careful consideration, our cases suggest that with appropriate planning, these patients can undergo complex cardiac procedures safely.