Medical Without life-prolonging interventions,<unk> S is fatal, but with intervention, an infant may survive. A cardiothoracic surgeon may perform a series of operations or a full<unk>  transplant. While surgical intervention has emerged as the standard of care in the United States, other national<unk> h systems, notably in France, approach diagnosis of<unk> S in a more conservative manner, with an emphasis on termination of pregnancy or compassionate care after delivery. Before surgery, the ductus must be kept open to allow blood-flow using medication containing prostaglandin. Air with less oxygen than normal is used for infants with<unk> . These low oxygen levels increases the pulmonary vascular resistance (PVR) and thus improve blood flow to the rest of the body due to the greater pressure difference between the lungs and body. Achieving oxygen levels below atmosphere requires the use of inhaled nitrogen. Nitric oxide is a potent pulmonary vasodilator, and thus reduces PVR and improves venous return. Any factor that increases PVR will impede right sided flow. Surgical Surgical operations to assist with<unk> <unk><unk>  are complex and need to be individualized for each patient. A cardiologist must assess all medical and surgical options on a case-by-case basis. Currently, infants undergo either the staged reconstructive surgery (Norwood or Sano procedure within a few days of birth, Glenn or Hemi-Fontan procedure at 3 to 6 months of age, and the Fontan procedure at 1 1/2 to 5 years of age) or cardiac transplantation. Current expectations are that 70% of those with<unk> S may reach adulthood. Many studies show that the higher the volume (number of surgeries performed) at a hospital, the lower the mortality (death) rate. Factors that increase an infant's risk include lower birth weight, additional congenital anomalies, a genetic syndrome or those with a highly restrictive atrial septum.) For patients without these additional risk factors, 5 year survival now approaches 80%. Studies show that about 75% of those children who survive surgery show developmental delays in one or more areas, such as motor, cognitive, or language impairments, with about a third of single-ventricle children without a genetic syndrome having significant impairments. Current research focuses on charting the connections between neurodevelopment injuries, surgical and intensive care procedures, and genetic susceptibility with the goal of modifying interventions that impair neurodevelopmental and psychosocial outcomes. An alternative to the traditional Norwood is the Hybrid procedure. Some physicians offer compassionate care, instead of the surgeries, which results in the child's death, usually within 2 weeks of birth. Compassionate care is overseen by a physician, and may be carried out either in the hospital or at home. However, due to the vast improvement of surgical intervention, with many hospitals achieving over 90% survival, there is debate on whether or not compassionate care should still be offered to families. A study in 2003 concluded that a selection of physicians who are experts in the care of children with<unk> S were evenly split when asked what they would do if their own children were born with<unk> S, with 1/3 stating that they would choose surgery, 1/3 stating that they would choose palliative (compassionate) treatment without surgery, and 1/3 stating that they are uncertain which choice they would make. The three-stage procedure is a palliative procedure (not a cure), as the child's circulation is made to work with only two of the<unk> 's four chambers. Norwood procedure Norwood procedure The first step is the Norwood procedure. In this procedure, the right ventricle is used to pump blood into the systemic circulation. Since the right ventricle is no longer directly pumping blood to the lungs, a shunt is required in order to pass deoxygenated blood through the lungs. Either the subclavian artery can be connected to the pulmonary circulation (Blalock-Taussig shunt), or a shunt is made directly from the right ventricle to the pulmonary circulation (Sano shunt). The narrow aorta is enlarged using a patch to improve blood flow to the body. During this time the baby may be medically fragile and have feeding problems because the<unk>  is working very hard. There is a considerable degree of venous mixing in the right ventricle, leading to lower oxygenation saturation. In addition, both the Blalock-Taussig and the Sano shunts expose the lungs to systemic arterial pressures, leading to long-term pulmonary hypertension and eventually<unk>  failure. Hybrid procedure The Hybrid procedure may be used in place of the Norwood. The Hybrid procedure does not necessitate the use of<unk> -lung bypass or performing a sternotomy. Instead of a six-hour surgery, the Hybrid typically takes one to two hours. In this procedure, a stent is placed in the ductus arteriosus to maintain its patency, and bands are placed over both the<unk>  and right pulmonary artery branches to limit pressure and over-circulation to the lungs. Outcomes with the Hybrid approach are comparable to those with the Norwood. Glenn procedure The second stage—the bidirectional Glenn or Hemi-Fontan (see also Kawashima procedure)—relieves some of the problems introduced by Stage I palliation. In this operation, the superior vena cava is ligated from the<unk>  and connected to the pulmonary circulation. At this time, the Blalock-Taussig or Sano shunt is taken down. The lungs are no longer exposed to systemic arterial pressures, but much lower venous pressures. Although venous blood from the upper half of the body is no longer mixing with oxygenated blood in the right ventricle, there is still venous mixing from the lower half of the body, leading to some degree of oxygen desaturation. Fontan procedure upright=1.5 The final procedure, the Fontan procedure, completes the repair of the<unk> <unk><unk> . Although there are several variations, the functional effect is to redirect venous blood from the lower body (through the inferior vena cava) away from the right atrium to the pulmonary artery. This should eliminate any mixing of oxygenated and deoxygenated blood in the right ventricle. The right ventricle performs the traditional job of the<unk> , supplying the body with oxygenated blood, while the passive systemic venous pressure performs the traditional job of the right, passing deoxygenated blood to the lungs. Fetal surgery Interventions performed during fetal development are under investigation. In fetuses with<unk> <unk>  ventricles and an intact interatrial septum, percutaneous atrial septostomy has been attempted.
Hypoplastic left heart syndrome
Hypoplastic right heart syndrome
Asphyxiating thoracic dysplasia
Double aortic arch
Pulmonary atresia
Double outlet right ventricle
Pulmonary hypoplasia
Congenital diaphragmatic hernia