Holt-Oram syndrome with double outlet right ventricle with valvular and subvalvular pulmonary stenosis

The Holt-Oram syndrome is an autosomal dominant condition characterized by skeletal abnormalities that are frequently accompanied by congenital cardiac defects, most commonly an atrial septal defect. We report a rare case of sporadic Holt-Oram syndrome with double outlet right ventricle [DORV], valvular and subvalvular pulmonary stenosis, persistent left superior vena cava draining to the left atrium, and an ectopic right kidney

Early extubation in pediatric patients after cardiothoracic surgery

Early extubation after cardiac operations is an important aspect of fast-track cardiac anesthesia. In order to reduce or eliminate the adverse effects of prolonged ventilation, the concept of early extubation in pediatric patients has been examined at our institution. To allow rapid emergence from anesthesia post cardiac surgery, low-dose opioids, supplemented with continuous propofol infusion and low concentration of inhaled agent was used. Intercostal nerve block was used in thoracotomy operations. Contraindications to early extubation were: cardiopulmonary bypass [CPB] > 2.5 hours, hemodynamic instability, uncontrolled bleeding, severe pulmonary hypertension and congestive heart failure. Eighty-two consecutive patients were reviewed. The age range was 6 months - 14 years with mean of 3.3 years. Closed cardiac procedures were performed in 15 [18.3%] patients, and operations with Cardio-pulmonary bypass in 67 [81.7%] patients. No patient required re-intubation during the first 24 hours after operation. One patient was re-intubated 48 hours after extubation for sputum retention. There was no mortality, and the incidence of perioperative morbidity was low. Early extubation after pediatric cardiothoracic operations can be achieved safely, and is possible in the majority of such patients