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
o
o
o
o
o

cyanosis
tachycardia
pounding heart
cold extremities
rapid breathing
lethargy

Background
Frequency:
o

2-3% of all congenital heart defects

2-3 cases per 10,000 live births

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

if left untreated, HLHS is responsible for 25-40% of

all neonatal cardiac death

Background
Risk factors:
o

More common in males than females

Environmental teratogens

Etiology: no known cause

Interventions: Non-surgical
Medications are used to strengthen the heart
and lower the hearts 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 babys 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 oxygenpoor 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
o

As infant and toddler

Possible surgeries in 20s & 30s

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
http://www.cdc.gov/ncbddd/heartdefects/hlhs.html
MedlinePlus Medical Encyclopedia. (2008). Hypoplastic left heart syndrome. Retrieved from
http://www.nlm.nih.gov/medlineplus/ency/article/001106.htm
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
http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002096/#adam_001106.disease.causes
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), 46-66. Retrieved from
http://ccn.aacnjournals.org/content/24/6/46.