Life-saving heart surgery is always required. If the diagnosis is made in a standard hospital or other clinical facility, the baby will be transferred to a children's hospital, if such facilities are available, for specialized paediatric treatment and equipment. The patient will require constant monitoring and care in an intensive care unit (ICU). Palliative Palliative treatment is normally administered prior to corrective surgery in order to reduce the symptoms of<unk>  (and any other complications), giving the newborn or infant a better chance of surviving the surgery. Treatment may include any combination of: Surgery Minor * Cardiac catheterization * Rashkind balloon atrial septostomy * Balloon angioplasty * Endovascular stenting * Angiography Cardiac catheterization is a minimally invasive procedure which provides a means of performing a number of other procedures. A balloon atrial septostomy is performed with a balloon catheter, which is inserted into a patent foramen ovale ( PFO ), or atrial septal defect ( ASD ) and inflated to enlarge the opening in the atrial septum; this creates a shunt which allows a larger amount of oxygenated ("red") blood to enter the systemic circulation. Angioplasty also requires a balloon catheter, which is used to stretch open a stenotic vessel; this relieves restricted blood flow, which could otherwise lead to congestive heart failure (CHF). An endovascular stent is sometimes placed in a stenotic vessel immediately following a balloon angioplasty to maintain the widened passage. Angiography involves using the catheter to release a contrast medium into the chambers and/or vessels of the heart; this process facilitates examining the flow of blood through the chambers during an echocardiogram, or shows the vessels clearly on a chest x-ray, MRI, or CT scan - this is of particular importance, as the coronary arteries must be carefully examined and "mapped out" prior to the corrective surgery. It is commonplace for any of these palliations to be performed on a<unk>  patient. Moderate * Left anterior thoracotomy * Isolated pulmonary artery banding (PAB) * Left lateral thoracotomy * PAB (when coarctation or aortic arch repair also required) * Right lateral thoracotomy * Blalock-Hanlon atrial septectomy Each of these procedures is performed through an incision between the ribs and visualized by echocardiogram; these are far less common than heart cath procedures. Pulmonary artery banding is used in a small number of cases of<unk> , usually when the corrective surgery needs to be delayed, to create an artificial stenosis in order to control pulmonary blood pressure; PAB involves placing a band around the pulmonary trunk, this band can then be quickly and easily adjusted when necessary. An atrial septectomy is the surgical removal of the atrial septum; this is performed when a patent foramen ovale ( PFO ), or atrial septal defect ( ASD ) are not present and additional shunting is required to raise the oxygen saturation of the blood flowing eventually into the aorta. Major * Median sternotomy * PAB (when intracardiac procedures also required) * Concomitant atrial septectomy In recent years, it is quite rare for palliative procedures to be done via median sternotomy. However, if a sternotomy is required for a different procedure, in most cases all procedures that are immediately required will be performed at the same time. Monitoring and maintenance Palliated pre-operative<unk>  + VSD neonate. * Nasogastric tube (NG tube or simply NG) * Intubation, oxygen mask, or nasal cannula * Intravenous drip (IV) * Arterial line * Central venous catheter * Fingerprick * Sphygmomanometer * Pulse oximeter * EKG An NG tube is used to deliver nourishment, and occasionally medication, to the patient. Since the tube extends right into the stomach, it can also be used to monitor how well the patient is digesting their "food". Paediatric units normally provide facilities and equipment for mothers of infant patients to pump their breastmilk, which can then be fed to the infant through the NG tube, and/or stored for later use. Oxygen therapy is commonplace for hospitalized<unk>  patients. This may range from an oxygen mask resting on the bed nearby their head to intubation. In some cases, patients are intubated as a precaution; the machine can monitor breathing and supplement the patient as much or as little as they need. IV's are used to deliver medication, blood products, or other fluids to the patient. Arterial lines provide a constant monitor of blood pressure, as well as a method of obtaining samples for blood gas tests; central lines can also monitor blood pressure and provide blood samples, as well as provide a means to deliver medication and nourishment; fingerpricks (or heelpricks on small babies) are used to obtain blood samples for certain tests. A sphygmomanometer may be used for intermittent blood pressure monitoring even if a patient is being otherwise monitored using a central or arterial line. A pulse oximeter is attached to a finger or toe and provides constant or intermittent monitoring of the blood's oxygen saturation level. An EKG creates a visual readout of how well the heart rhythm is functioning. Medication * Prostaglandin (PGE) * Antibiotics * Diuretics * Analgesics * Cardiac glycosides * Sedatives When PGE is administered to a newborn, it prevents the ductus arteriosus from closing, therefore providing an additional shunt through which to provide the systemic circulation with a higher level of oxygen. Antibiotics may be administered preventatively. However, due to the physical strain caused by uncorrected<unk> , as well as the potential for introduction of bacteria via arterial and central lines, infection is not uncommon in pre-operative patients. Diuretics aid in flushing excess fluid from the body, thereby easing strain on the heart. Analgesics normally are not used pre-operatively, but they may be used in certain cases. They are occasionally used partially for their sedative effects. Cardiac glycosides are used to maintain proper heart rhythm while increasing the strength of each contraction. Sedatives may be used palliatively to prevent a young child from thrashing about or pulling out any of their lines. Corrective surgery Nikaidoh Bex who introduced the possibility of aortic translocation in 1980. But Nikaidoh has put the procedure in practice in 1984. It results in an anatomical normal heart, even better than with an ASO, because also the cones are switched instead of only the arteries as with an ASO. The procedure is contra-indicated by certain coronary anomalies. In 1984, Nikaidoh introduced a surgical approach for the management of TGA, VSD, and pulmonary stenosis (PS), which he called “aortic translocation and biventricular outflow tract reconstruction.” The repair consisted of harvesting the aortic root from the right ventricle, with or without the coronary arteries attached, and relieving the LVOTO by dividing the outlet septum and pulmonary valve annulus. The LVOT is then restored by posteriorly translocating the aortic root and closing the VSD. Finally, the right ventricular outflow tract is reconstructed with a pericardial patch. This is a technically challenging procedure, but results in a more “normal” anatomic repair. The main thing is the repositioning of the native aortic root over to the LV cavity, avoiding the creation of a long tortuous intraventricular tunnel. This technique appears to prevent the development of LVOTO, which is a frequent complication of the Rastelli repair. The addition of the Lecompte maneuver may prevent branch pulmonary artery stenosis that may occur secondary to compression of the PA by the posteriorly displaced, translocated aortic root. It creates a direct RV to PA anastomosis and avoiding the use of a conduit, which should decrease the incidence of RVOT reinterventions. Lecompte Since 1981 LeCompte has put his LeCompte manoeuvre in use. This is used with the REV (Réparation à l'Etage Ventriculaire). This surgery is like the Rastelli procedure, but with the use of the pulmonary artery without a conduit. Rastelli When an arterials switch operation (ASO) is not possible e.g. in case of LVOTO an option is the Rastelli procedure. The pulmonal artery is shifted with help of conduit to the right ventricle. It has been used since 1960s. It has a disadvantage that the conduit does not grow, so re-operation is necessary. Arterial switch Arterial switch procedure. Immediate post-operative (Jatene procedure)<unk>  + VSD neonate. The Jatene procedure surgery is the preferred, and most frequently used, method of correcting<unk> . It is ideally performed on an infant between 8–14 days old. The heart and vessels are accessed via median sternotomy, and a cardiopulmonary bypass machine is used; as this machine needs its "circulation" to be filled with blood, a child will require a blood transfusion for this surgery. The procedure involves transecting both the aorta and pulmonary artery; the coronary arteries are then detached from the aorta and reattached to the neo-aorta, before "swapping" the upper portion of the aorta and pulmonary artery to the opposite arterial root. Including the anaesthesia and immediate post operative recovery, this surgery takes an average of approximately six to eight hours to complete. Some arterial switch recipients may present with post-operative pulmonary stenosis, which would then be repaired with angioplasty, pulmonary stenting via heart cath or median sternotomy, and/or xenograft. One day
Dextro-Transposition of the great arteries
Double aortic arch
Levo-Transposition of the great arteries
Transposition of the great vessels
Double outlet right ventricle
Tetralogy of Fallot
Trilogy of Fallot
Interrupted aortic arch