What Are the Long-term Effects of Hypoplastic Left Heart Syndrome?
Left untreated, children with hypoplastic left heart syndrome usually die in early infancy. The long-term outlook is only beginning to be known, as this heart defect used to be fatal.
Virtually every child with this condition will require heart medicines. Depending on the success of surgery, individuals may lead functional lives for the rest of their days. Over time, the function of the right ventricle may worsen, and heart transplantation may be needed.
Children with hypoplastic left heart syndrome require lifelong follow-up appointments with a pediatric cardiologist and ultimately transition to an adult congenital heart disease specialist.
Problems encountered over time, or known morbidities include:
- Congestive heart failure
- Protein loosing enteropathy (PLE)
- Plastic bronchitis
- Fontan-related liver disease
- Chronic kidney disease
- Endocrine abnormalities (bone disease, growth failure)
- Thromboembolic events
- Neurodevelopmental concerns
Interstage Home Monitoring (IHM) Program
Research has shown that it is critically important to have an interstage home monitoring (IHM) program, that includes daily, at home assessments of oxygen saturations and weight between the stage 1 and stage 2 surgeries. Patients in an HMP are normally infants – the stage 2 repair, a Glenn, is typically performed between four- and six-months of age.
Daily home pulse oximeter checks alert the team of any sudden or changing patterns in the oxygenation. Daily weight checks tell parents and providers if a child is dehydrated, makes quick medical intervention possible, and decreases the rate of sudden death in this very fragile population of infants.
During this period, the primary caregiver at home, Single Ventricle Program APN, primary cardiologist and pediatrician will need to be in close communication regularly. This can be in the clinic and by phone. These frequent check-ins will help the care team catch any subtle changes in your baby’s health.
Parents have been instructed to call if any one of these red flags is seen at home:
- Your child’s oxygen saturations change by:
- Dropping consistently below 75%
- Increase suddenly more than 90%
- Your child is breathing harder, faster or is fussy.
- Your child is unable to meet maintenance goal volume for 24 hours.
- Your child is unable to attain goal to grow volume/24h for more than three days.
- Your child does not gain .02 Kg or 20 grams over a 3-day span.
- Your child loses .03kg or 30 grams or more over a 2–3 day span.
- Your child has a temperature of 100.5 or greater.
- You have any other questions or concerns.
Questions & Concerns
If you have any questions or concerns after your child is discharged, our team is available to take your call.
Mondays through Fridays, 8 a.m.-4 p.m., call the single ventricle nurse practitioner at 312.227.4000 and ask for Michelle Steltzer, CPNP-AC/PC.
Mondays through Fridays after 4 p.m. and on weekends, call the hospital operator at 312.227.4000 and ask for the cardiology fellow on call.