Download Hypoplastic Left Heart Syndrome

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
cyanosis
o tachycardia
o pounding heart
o cold extremities
o rapid breathing
o lethargy
o
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 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 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
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.