Hypoplastic Left Heart Syndrome By: Tyler Nickels, Amanda McKellar, Kassie Herp, Zachary Zwiernikowski, & Amanda Mathy

Background ● Congenital defect that occurs when parts of the left side of the heart do not develop completely. - Examples: mitral valve, aorta, aortic valve, left ventricle ● Right side must compensate by providing body & lung circulation; eventually right side fails. ● Foramen ovale must be kept open to maintain adequate circulation

Background ● Symptoms are similar to anyone with oxygen depletion o cyanosis o tachycardia o pounding heart o cold extremities o rapid breathing o lethargy

Background ● Frequency: o 2-3% of all congenital heart defects o 2-3 cases per 10,000 live births o most common form of functional single ventricle heart disease

Background ● Significance o about 10% of infants born with this condition also have other birth defects o if left untreated, HLHS is responsible for 25-40% of all neonatal cardiac death

Background ● Risk factors: o More common in males than females o Environmental teratogens o Etiology: no known cause

Interventions: Non-surgical ● Medications are used to strengthen the heart and lower the heart’s physical workload o Prostaglandin E1 (Misoprostol)  Vasodilation ● Nutrition is regulated with high-calorie formulas

Interventions: Surgical ● Surgeries do not cure HLHS, but can help to restore heart function. ● Multiple surgeries are needed before blood flow can be properly bypassed past the poorly functioning parts of the heart.

Interventions: Norwood Procedure Occurs within first 2 weeks of baby’s life ● Creation of new aorta and connection to right ventricle ● Reroute blood flow from right side of heart to pulmonary arteries ● Baby may look blueish after procedure due to the mixing of oxygen-rich and oxygen- poor blood

Interventions: Bi-direction Glenn Shunt Occurs at 4-6 months of age ● Creates direct connection between pulmonary artery and superior vena cava to return oxygen-poor blood from upper part of body to the heart

Interventions: Fontan Procedure Occurs between 18 months and 3 years of age ● Connect pulmonary artery and inferior vena cava ● Allows rest of blood coming from lower part of body to go to the lungs ● Oxygen-rich and oxygen-poor blood no longer mixes in heart

Interventions: Post-surgical ● Infants may still have lifelong complications, even after successful surgeries ● Regular follow-up visits with a cardiologist are required to monitor progress ● Heart transplants may be needed if heart becomes weak after surgery

Diagnostic Procedures ● Physical exams o Elevated HR & breathing, weak pulses, abnormal heart sounds ● Cardiac Catheterization ● EKG ● Echocardiogram ● X-ray of chest

Treatment ● NICU at birth o Ventilator may needed ● Medication: Prostaglandin E1 (Misoprostol) o Vasodilator of the ductus arteriosus ● Surgery o As infant and toddler o Possible surgeries in 20’s & 30’s ● Transplant o alternative (?) to 3-step surgery

Nursing Role: Care of patient ● Consistency ● Nurse to Nurse Report ● Assessment ● Monitor response ● Intracardiac catheters

Nursing Role: Care of parents ● Educate ● Informed decision ● Relate ● Support

Nursing Role: Nursing Diagnosis ● Delayed Growth and Development r/t inadequate oxygen and nutrients to tissues ● Decreased Cardiac Output r/t structural alterations

Nursing Role: Interventions r/t diagnosis ● Oxygen Therapy ● Family coping ● Monitor lung sounds for pulmonary edema ● Semi-fowlers position ● Closely monitor fluid intake ● Sodium restricted diet ● Provide normal sleep and wake times to promote growth and development

Expected Outcomes: Mother -Medication Administration -Doctors Appointments -Coping with stress

Expected Outcomes: Baby -75% -Heart transplant -Surgery in 20s-30s

References CDC. (2014). Facts about hypoplastic left heart syndrome. Retrieved from MedlinePlus Medical Encyclopedia. (2008). Hypoplastic left heart syndrome. Retrieved from Ladwig, G. B., & Ackley, B. J. (2014). Guide to Nursing Diagnosis (4th ed.). Maryland Heights, MO: Elsevier. Marshall, A. (2015, January 22). Hypoplastic left heart syndrome. In Wolters Kluwer Health. NCBI. (2013, November 5). Hypoplastic left heart syndrome. In PubMed Health. Retrieved from Soetenga, D., & Mussatto, K. A. (2004, December). Management of infants with hypoplastic left heart syndrome integrating research into nursing practice. Critical Care Nurse, 24(6), Retrieved from