Hypoplastic Left Heart Syndrome is a life threatening congenital cardiac anomaly. After a child has been diagnosed with hypoplastic left heart syndrome, parents must make life or death decisions within days of birth. Healthcare providers must provide appropriate education so that parents are able to make informed, timely decisions. Information regarding the diagnosis, treatment options, and parental decision making process for initial decision making for hypoplastic left heart syndrome are provided to guide nurses who work with these families. The challenging decision making process which parents must go through after diagnosis of hypoplastic left heart syndrome will be described.
Rachel and Evan, a young couple expecting their first child, received unexpected and devastating news at the completion of a routine screening ultrasound at 20 weeks. The ultrasound revealed that their unborn son had a congenital heart defect. After further testing, the diagnosis of hypoplastic left heart syndrome (HLHS) was confirmed. Rachel and Evan received information about HLHS from several physicians, made an appointment with a pediatric cardiothoracic surgeon to discuss surgical options, and found information from pediatric hospitals and HLHS online support groups. Ultimately, Rachel and Evan had decisions to make. Four options were presented by the healthcare team, including (1) terminating the pregnancy immediately, (2) continuing with the pregnancy and proceeding with staged palliative surgical intervention in the first week of their son’s life, (3) continuing with the pregnancy with the hopes of a heart transplant in the first week of their son’s life, or (4) choosing comfort care after delivery, and allowing death to occur naturally, likely in the first week of life. The time frame for option one was urgent because of abortion laws in their state and the gestational age of the fetus. After several sleepless nights of reviewing information, statistics, options, and prognosis, they elected to continue with the pregnancy and give their son a chance at life.
Date of this Version
Toebbe, Sarah; Yehle, Karen S.; Kirkpatrick, Jane; and Coddington, Jennifer, "Hypoplastic Left Heart Syndrome: Parent Support for Early Decision Making" (2012). School of Nursing Faculty Publications. Paper 18.
Karen Yehle, (2012), Hypoplastic left heart syndrome: Parent support for early decision-making, Journal of Pediatric Nursing, Vol 28, Issue 4, 383-392, 10.1016/j.pedn.2012.11.005. NOTICE: this is the author’s version of a work that was accepted for publication in Journal of Pediatric Nursing. Changes resulting from the publishing process, such as peer review, editing, corrections, structural formatting, and other quality control mechanisms may not be reflected in this document. Changes may have been made to this work since it was submitted for publication. A definitive version was subsequently published in Journal of Pediatric Nursing, [Vol 28, Issue 4, 383-392], (2012), 10.1016/j.pedn.2012.11.005.