Hypoplastic left heart syndrome

Hypoplastic left heart syndrome (HLHS) is one of the last remaining problems in pediatric cardiac surgery, which necessitates a search for new solutions and continues to be a challenge for cardiologists and cardiac surgeons. The identification of HLHS in utero is extremely helpful for prompt, appropriate preoperative management. In addition, infants with prenatal diagnosis had an improvement operative mortality on Norwood operation compared to infants diagnosed postnatally. Outcome following Norwood procedure has improved significantly secondary to modifications in the surgical technique for protection of multiorgans, improved perioperative care, and improved anesthetic management. In particular right ventricular (RV)-pulmonary artery (PA) shunt using non-valved polytetrafluoroethylene (PTFE) graft was applied as a first stage palliation of HLHS to prevent hemodynamic instability associated with a classic Norwood procedure. In the modified Norwood (RV-PA shunt), higher diastolic blood pressures and lower Qp/Qs ratios that are associated with a more stable and efficient systemic circulation. In fact, patients who received a RV-PA shunt, rarely received ventilatory manipulations.