Single ventricle repair

A more complete repair depends on the size of the pulmonary artery and right ventricle. If the pulmonary artery and right ventricle are very small, the patient may require the same type of operation as other single ventricle patients. In some patients, abnormal channels (sinusoids) form between the coronary. To repair many types of single ventricle heart defects , including hypoplastic left heart syndrome (HLHS ), tricuspid atresia , double outlet left ventricle (DOLV ), some heterotaxy defects , and other congenital heart defects , surgeons often perform a series of open heart procedures over several years.

This is called “staged reconstruction. Single ventricle defects require a series of open heart procedures , performed over several years.

Surgeons reconfigure the heart and circulatory system during the procedures. The single ventricle must not be overworked for a long period of time , in terms of either having to pump too much blood or pump at too high a pressure. The pulmonary arteries must grow well without stenosis (narrowing) and must remain low resistance (or be very relaxed). They are also one of the most complex heart problems, usually requiring at least one surgery.

There are several types of single ventricle defects. Most babies with a single ventricle heart defect have a series of three heart surgeries , called single ventricle palliation, that culminate in the Fontan procedure. These surgeries allow the one working ventricle to do the work of two ventricles.

What is the Glenn procedure? Background: Dextrocardia is found in a significant proportion of patients undergoing a single-ventricle repair.

Surgical outcomes in this cohort are unclear. An alternative, single ventricle, pathway has been proposed for any patient without optimal preoperative anatomy to improve long-term survival. Adjunctive repair options including pulmonary artery banding and one-and-a-half ventricle repairs have also been proposed to augment the survival curves.

The Fontan procedure is used in pediatric patients who possess only a single functional ventricle , either due to lack of a heart valve (e.g. tricuspid or mitral atresia), an abnormality of the pumping ability of the heart (e.g. hypoplastic left heart syndrome or hypoplastic right heart syndrome), or a complex congenital heart disease where a bi-ventricular repair is impossible or inadvisable. A complex single ventricle repair is tailored to the type of defect and many other factors. It takes experience, skill and training to match the right treatment with your child’s unique condition.

The Society of Thoracic Surgeons has rated Norton Children’s Heart Institute’s pediatric heart care among the best in the region. All forms of single ventricle are a staged repair with an initial neonatal repair as described above, followed by a Bidirectional Glenn at months and a Fontan (Bicaval pulmonary anastomosis) at 3-years of age. In this group, single ventricular palliation and ventricular recruitment with staged biventricular conversion was recommended. Staged surgery is usually started in the first week of life.

The surgeon will fix any other abnormalities, such as a faulty valve. Once all the repairs have been done and the heart is beating normally, the heart-lung machine will be removed. The breast bone will be put back together with wires.

Widen the narrowed pulmonary artery to allow more blood flow. Place an aorta to pulmonary artery shunt to provide more blood flow to the pulmonary arteries. Conduct a series of other procedures to allow blood to move to the lungs and for the heart to function with one ventricle , if the defect is complex.

The Norwood procedure involves atrial septectomy and transection and ligation of the distal main pulmonary artery. Eventually, all people with a single ventricle will need one or more surgeries which allow the blue blood to drain to the lungs by gravity, flow to the single ventricle , and get pumped to the body.

In a single ventricle heart, only one chamber is large enough to pump all of the blood. The proximal pulmonary artery is then connected to the hypoplastic aortic arch, while the coarcted segment of the aorta is repaired. It is important that it is used efficiently and not overworked. With single ventricle physiology, there are usually at least three operations performed. The goal of the series of surgeries (called “single ventricle palliation”) is to transform the heart and circulation from the abnormal circulation at birth to one where the heart: pumps the blood out to the body.

The surgical treatment of single ventricle is based on the severity of the condition. Infants are usually treated with staged surgical intervention beginning in the first week of life. The first procedure is called a shunt. The shunt helps to increase blood flow to the lungs. The treatment of single ventricle depends on the particular defect.

It is necessary to separate the pulmonary (heart to lung) and systemic (heart to body) circulations so that oxygen-rich blood can be pumped to the body tissues. Fontan pathways preserve some of the pressure energy in the aorta and make it available to improve filling (preload) of the systemic ventricle. In single ventricles filling from the systemic and pulmonary veins, the filling pressure must be low enough to accomodate the systemic veins.

The Rastelli operation was initially utilized for the repair of d-transposition of the great vessels with ventricular septal defect and pulmonary stenosis. It has subsequently been utilized for a variety of congenital heart defects characterized by two ventricles and overriding of the aorta with severe pulmonary stenosis or pulmonary atresia.