Hypoplastic Left
Heart Syndrome
Image 1:
Image courtesy of Koji Kagasaki, MD. Image found on the website: http://emedicine.medscape.com/article/904137-treatment#a1133
*Cardiopulmanary Bypass: This is a procedure that temporarily takes over the function of the heart and lungs during surgery. It maintains the circulation of blood and the oxygen content of the body and is sometimes called the heart-lung machine.
Steps of the Glenn Procedure
(These steps may not be conducted in this order and there are smaller steps that are taken between the main steps below.)
1) Cardiopulmonary bypass* (CBP) is achieved
2) The aortopulmonary shunt that is there is closed and divided when CPB is started.
3) If there is any narrowing of the pulmonary artery close to the prior shunt or patch, it is repaired.
4) The azygous vein is closed and divided.
5) The Superior Vena Cava is cut and is connected in an end-to-side fashion to the upper part of the right pulmonary artery. The cardiac end of the Superior Vena Cava is then is oversewn. Some groups routinely perform the bidirectional Glenn procedure without CPB and with or without an Superior Vena Cava-to–right atrial temporary shunt during the anastomosis to lower the high cerebrovenous pressures (pressure of blood in the vena cava near the right atrium of the heart).
6) The aortopulmonary shunt is divided, and the pulmonary arteries are moved from the right to the left upper lobe.
7) The azygous vein is closed and the right atrium is opened along the upper part. An incision is made transversely along the junction of the branch of the pulmonary arteries (see upper left of Image 1).
8) The pulmonary arteries are connected to the upper portion of the right atrium along the incisions (see upper right of Image 1)
9) A patch of pulmonary allograft tissue is used to add to the pulmonary arteries and support them. The allograft patch is begun at the left upper lobe, incorporating a separate end-to-side connection for a left Superior Vena Cava, if necessary (see lower portion of Image 1).
10) A patch is placed within the right atrium, which has several functions such as providing an unobstructed pathway for connection of Inferior Vena Cava during the Fontan procedure (see lower portion of Image 1).
11) The atrial septal defect is inspected and enlarged, if necessary, which is completed best by cutting back the coronary sinus (a wide vein in length that receives blood from the coronary veins and empties into the right atrium) into the left atrium.
12) Tricuspid valve repair is also performed as needed.