It is the philosophy of our institution to provide primary surgical repair of complex congenital heart defects to avoid pulmonary and myocardial damage that occurs over time. Application of knowledge of the normal physiology and a clear understanding of the physiologic alterations of complex lesions are essential in understanding the effect of positive-pressure ventilation in complex CHD. The care and management of patients with CHD have a great deal to do with ventilatory management, which can be life-threatening when not fully understood but lifesaving when mastered. A dedicated team approach enhances communication and collaborative practice, which provides optimal patient management and outcome.