Surgical treatment concepts for end-stage congenital heart diseases

It is anticipated that as many as 10-20% of patients alive with anatomical congenital heart lesions may eventually develop heart failure. Most of these patients have undergone previous palliative or corrective surgeries. The Fontan procedure, although it has helped many patients with single-ventricle physiology to become hemodynamically functional, it is associated with protein-losing entropathy, intractable atrial arryhythmia and systemic ventricular dysfunction. In patients with transposition of the great arteries, physiological or intra-atrial repair techniques (Mustard and Senning) or anatomical correction (arterial switch) have been used. The majority of these patients are clinically well throughout their adult life but, as a consequence of the potential for right ventricular failure, some may develop cardiac insufficiency. Although long-term survival and quality of life in children and adults with complex congenital heart disease have remarkably improved due to advances in operative techniques and perioperative management, as well as the increasing experience of congenital heart surgeons, a growing number of these patients eventually develop end-stage heart failure and will require another treatment. Surgical options for treatment of these patients are limited. Heart transplantation has become a well-established treatment option for children as well as adults with end-stage congenital heart disease. Because of organ donor shortage, another option is mechanical circulatory assist device implantation, either as a bridge to transplantation, or as a permanent therapy.