Summer 2008

Case Study: HLH Syndrome

JS was born nine months ago with hypoplastic left heart syndrome (HLHS), a congenital heart condition. To completely palliate JS' HLHS, three procedures would be required: stage I (Norwood procedure), performed at birth; stage II (bidirectional cavopulmonary anastomosis), performed at 4 to 6 months of age; and stage III (Fontan procedure), performed at 4 to 5 years of age.

Referral to UCSF Benioff Children's Hospital

Although HLHS is a very complex congenital heart defect and is considered by most physicians to be fatal, UCSF's pediatric cardiac surgery team has a 90 percent to 95 percent survival rate with the stage I procedure. JS' diagnosis was made prenatally by a community cardiologist, and his mother was referred to UCSF. JS' prenatal diagnosis was especially helpful for optimal management of his circulation so that he could be in good condition for surgery.


The multidisciplinary evaluation and diagnostic testing performed at UCSF included imaging using echocardiography and cardiac catheterization, as well as advanced magnetic resonance imaging and electrophysiology.


JS had his stage I palliation soon after birth and recovered. The stage I procedure involved reconstruction of the aortic arch and attachment of a Gore-Tex shunt from the single right ventricle to the pulmonary artery, to provide JS with a source of blood flow to the lungs.

After the first procedure, JS went home for several weeks, and then returned for his stage II palliation. Since the Gore-Tex shunt does not grow with the child, a secondary source of pulmonary blood flow was provided at the stage II operation. At that time, physicians connected the superior vena cava to the pulmonary artery, thereby enabling blue blood to get into the lungs to be oxygenated. In addition, repair of JS' tricuspid valve was done at that time.


Following his second surgery, JS recovered well and was discharged. His follow-up will include close monitoring by the cardiac team at UCSF Benioff Children's Hospital, as well as his referring physician.

For more information, contact the Pediatric Heart Center at (415) 353-2008

Related Information

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